PROFILE OF UVEITIS IN A TERTIARY CARE CENTRE IN

SOUTH INDIA

DISSERTATION SUBMITTED TOWARDS FULFILLMENT OF

THE RULES AND REGULATIONS FOR M.S. BRANCH III
OPHTHALMOLOGY EXAMINATION OF THE TAMILNADU
DR. M.G.R. MEDICAL UNIVERSITY TO BE HELD IN MAY 2018
 PROFILE OF UVEITIS IN A TERTIARY CARE CENTRE IN

SOUTH INDIA

SUBMITTED BY
DR. LIBIN SAM BABY
CHRISTIAN MEDICAL COLLEGE, VELLORE

DISSERTATION SUBMITTED TOWARDS FULFILLMENT OF

THE RULES AND REGULATIONS FOR M.S. BRANCH III
OPHTHALMOLOGY EXAMINATION OF THE TAMILNADU
DR. M.G.R. MEDICAL UNIVERSITY TO BE HELD IN MAY 2018
 BONAFIDE CERTIFICATE

This is to certify that this dissertation entitled ‘Profile of uveitis in a tertiary care centre in

South India’ done towards fulfillment of the requirements of the Tamil Nadu Dr. MGR Medical

University, Chennai, for the MS Branch III (Ophthalmology) examination to be conducted in

May, 2018, is the bonafide work of Dr. Libin Sam Baby, postgraduate student in the

Department of Ophthalmology, Christian Medical College, Vellore.

Dr. Anna Benjamin Pulimood, MD. Ph.D.

Principal
Christian Medical College
Vellore-632001
 BONAFIDE CERTIFICATE

This is to certify that this dissertation entitled ‘Profile of uveitis in a tertiary care centre in

South India’ done towards fulfillment of the requirements of the Tamil Nadu Dr. MGR Medical

University, Chennai, for the MS Branch III (Ophthalmology) examination to be conducted in

May, 2018, is the bonafide work of Dr. Libin Sam Baby, postgraduate student in the

Department of Ophthalmology, Christian Medical College, Vellore.

Dr. Andrew Braganza, M.S

Professor & Head of the Department
Department of Ophthalmology
Christian Medical College
Vellore-632001
 BONAFIDE CERTIFICATE

This is to certify that this dissertation entitled ‘Profile of uveitis in a tertiary care centre in

South India’ done towards fulfillment of the requirements of the Tamil Nadu Dr. MGR Medical

University, Chennai, for the MS Branch III (Ophthalmology) examination to be conducted in

May, 2018, is the bonafide work of Dr. Libin Sam Baby, postgraduate student in the

Department of Ophthalmology, Christian Medical College, Vellore.

Dr. Smitha Jasper, M.S, MPH

Associate Professor & Guide
Department of Ophthalmology
Christian Medical College
Vellore-632001
 BONAFIDE CERTIFICATE

I declare that this dissertation entitled ‘Profile of uveitis in a tertiary care centre in South India’

done towards fulfillment of the requirements of the Tamil Nadu Dr. MGR Medical University,

Chennai, for the MS Branch III (Ophthalmology) examination to be conducted in May 2018,

is the bonafide work of Dr. Libin Sam Baby, postgraduate student in the Department of

Ophthalmology, Christian Medical College, Vellore.

Dr. Libin Sam Baby

Postgraduate Student (MS Ophthalmology)
Registration Number: 221313304
Department of Ophthalmology
Christian Medical College
Vellore-632001
URKUND ORIGINALITY REPORT
 CERTIFICATE - II URKUND ORIGINALITY REPORT

This is to certify that this dissertation work titled ‘Profile of uveitis in a tertiary care centre in

South India’ of the candidate Dr. Libin Sam Baby with registration Number 221313304 for MS

Branch III (Ophthalmology) has been submitted for verification. I personally verified the

urkund.com website for the purpose of plagiarism Check. I found that the uploaded thesis file

contains from introduction to conclusion pages and result shows 1% percentage of plagiarism

in the dissertation

GUIDE
Dr. Smitha Jasper
Associate Professor
Department of Ophthalmology
 ACKNOWLEDGEMENT
All glory to God……

I express my sincere gratitude to all who contributed to this thesis:

Dr. Smitha Jasper, for her expert guidance from the conceptualization of the theme till the final

full stop; there is not one area of this thesis where her hands or mind have not touched.

Dr. Lekha Abraham, whose encouraging and reassuring words were the force that helped me

get the whole process started.

Mr. Bijesh for helping me with the statistics.

All the consultants for their constant encouragement.

My fellow registrars for being very helpful and considerate all throughout.

Mr. Deenadayalan, our librarian for all his help in providing various journals and books.

Mr. Anand and his Medical Records team for retrieving the charts whenever I needed for my

thesis.

My wife, Dr. Remya, our children Johanna and Joash and our parents for their constant support,

encouragement and prayers.

Last but not the least, I thank all my patients. Without them, my training would be incomplete

and this thesis would not have happened.

 TABLE OF CONTENTS

INTRODUCTION…………………………………………………………………1

AIM AND OBJECTIVES…………………………………………………………5

LITERATURE REVIEW………………………………………………………….6

MATERIALS AND METHODS…………………………………………………36

RESULTS AND ANALYSIS…………………………………………………….49

DISCUSSION…………………………………………………………………….78

LIMITATIONS OF THE STUDY………………………………………………..85

CONCLUSIONS………………………………………………………………….86

BIBLIOGRAPHY………………………………………………………………...89

ANNEXURES…………………………………………………………………….98
PROFILE OF UVEITIS IN A TERTIARY CARE CENTRE

IN SOUTH INDIA
INTRODUCTION

Inflammation of the uveal tract, the highly vascular middle layer of the eyeball (iris,

ciliary body, choroid) is termed Uveitis. Anatomically uveitis is classified as anterior,

intermediate, posterior or panuveitis, based on the anatomical parts of the uveal tract that is

predominantly involved. Anterior uveitis is the most common type of uveitis. It accounts for

50-92% of all uveitis cases in the West and 28-50% in the Asian countries. (1)

Uveitis is further classified based on clinical features, laterality, presentation and

etiology. Uveitis may present as an acute episode, recurrent episodes or remain a chronic entity

requiring systemic medication and resulting in many vision threatening sequelae. Uveitis may

present at any age and can affect both genders.

According to the appearance of keratic precipitates on the endothelial surface of cornea,

uveitis is classified as granulomatous or non-granulomatous. Laterality of involvement of the

eye has been described as unilateral, bilateral or alternating (affecting one eye at a time and

affecting both eyes alternately). (2) Etiologically, uveitis is classified as Infectious, Non-

infectious with a known systemic association or no known systemic association (idiopathic).(3)

Infectious cause of uveitis can be viral, bacterial, spirochetal and parasite related inflammation

and can present differently in immunocompetent and immunocompromised individuals.

Common viruses that cause uveitis are herpes simplex (HSV), herpes zoster (HZV),

cytomegalovirus (CMV), Epstein-Barr and human immunodeficiency virus (HIV). They are

more common in immunocompromised patients. However, recent studies of isolated anterior

uveitis have found HSV, VZV and CMV in the PCR of the aqueous samples. Herpes simplex

is the commonest virus that causes uveitis. (4) Acquired CMV infection occurs in 30% patients

with acquired immunodeficiency syndrome (AIDS), mostly as retinitis.

 Tuberculosis (TB) is a major cause of uveitis in developing countries. It can present as

acute, chronic or recurrent uveitis. Ocular manifestations may be caused by an active infection

that invades the eye or by a delayed type IV hypersensitivity immunological reaction to various

antigenic components of the mycobacteria, in the absence of the infectious agent. The clinical

manifestations of ocular TB vary depending on mycobacterial virulence, host resistance to

mycobacterium and the degree of tissue hypersensitivity to mycobacterial antigens. In India,

where pulmonary tuberculosis is endemic, incidence of TB as a cause among patients

presenting with uveitis is variable (0.6%-10.1%). The most common clinical presentations is

posterior uveitis, followed by anterior uveitis, pan uveitis, and intermediate uveitis (5–7)

Leprosy is caused by Mycobacterium leprae. About 60% of patients can develop ocular

manifestation and half of them develop uveitis, mostly bilateral disease. The commonest form

of anterior uveitis in leprosy is a chronic, low grade bilateral uveitis and usually becomes

symptomatic late in the disease process only. Cataract, synechiae, glaucoma, iris atrophy, and

ciliary body damage occurs insidiously causing loss of vision. As part of LOSOL study done

in Karigiri with 301 patients with multibacillary (MB) leprosy, the age-adjusted prevalence of

blindness and moderate to severe visual impairment was 2.8% and 5.2%, respectively. (8–10)

Syphilitic uveitis can be included in the differential diagnosis of any form of uveitis

due to its varied and unpredictable presentation. It can also occur at any stage of acquired

syphilis. The prevalence of syphilitic uveitis (9%) is now increasing again in view of the HIV

epidemic. (11)

Toxoplasma related retinochoroiditis is caused by Toxoplasma gondii, an obligate

intracellular parasite which causes progressive intraocular infection. This accounts for 30-55%

of posterior uveitis and is a major cause of visual impairment. (12)

 Non-infectious causes of uveitis are usually related to systemic connective tissue

disorders. The ocular symptoms and signs could present either before, during or after the onset

of systemic symptoms. The commonly encountered diseases are juvenile idiopathic arthritis,

HLA–B27 related disease, Behcet’s disease, rheumatoid arthritis, systemic lupus

erythematosus, Vogt-Koyanagi-Harada disease, sarcoidosis, Wegener’s granulomatosis, and

other undifferentiated connective tissue disorders.

HLA-B27 associated uveitis can be acute, anterior, non-granulomatous, recurrent and

unilateral alternating. Age of onset is usually in the third decade which is a decade earlier than

HLA-B27 negative anterior uveitis cases. HLA-B27 positivity rate between 41-56% has been

reported in India. Ocular involvement occurs in 25% of patients with ankylosing spondylitis.

(13)

Vogt-Koyanagi-Harada (VKH) disease, a severe bilateral diffuse non-necrotizing

granulomatous intraocular inflammation (posterior or panuveitis) associated with serous retinal

detachments, disc edema, and vitritis, with eventual development of a sunset glow fundus, is

an autoimmune inflammatory condition mediated by CD4+ T cells that target melanocytes in

individuals susceptible to the disease. VKH was the most common cause of panuveitis in India,

with a prevalence of 21.08 %.( 6)

Ocular inflammation occurs in about 70% in patients with Behcet’s disease and tends

to have a high risk of blindness. Disease tends to be more severe in men. Signs are always

bilateral eventually. Relapsing or remitting acute onset panuveitis with retinal vasculitis and

often spontaneous resolution even without treatment is the classical pattern of eye involvement.

(14)
 Sarcoid uveitis accounts for 3-10% of all cases of uveitis. Ocular inflammation occurs

in 25-70% of sarcoid patients depending on ethnicity. Granulomatous anterior uveitis is the

most common manifestation. (15–17)

Childhood uveitis is most commonly associated with juvenile idiopathic arthritis (JIA),

but can be observed in other autoimmune conditions like Behcet’s disease or sarcoidosis also.

(18)

Uveitis can have severe sequelae like posterior synechiae formation, cataract, cystoid

macular edema, glaucoma, visual impairment. Upto 25% of irreversible blindness in India and

other developing countries is due to uveitis. (19)

Early diagnosis, appropriate local and systemic treatment and follow up would

significantly reduce the morbidity associated with uveitis. Study of profile of uveitis in a

population gives the spectrum of disease, allows for prioritizing relevant investigations for the

region and can be used to develop management protocols and preventive measures as required

in the community. It helps to detect and monitor patterns of disease occurrence, changes over

time and their presenting patterns. There is no paucity of literature on uveitis. However, there

has not been a study on the profile of uveitis in patients seeking care for uveitis in South India

in the last 5 years.

 AIM AND OBJECTIVES
Aim:

To study the profile of uveitis in a tertiary care centre in South India

Objectives:

1. To study anatomic, demographic and etiologic profile of patients attending a uvea

clinic in a tertiary hospital in South India.

2. To study the characteristics, treatment response and complications of uveitis.

Review of Literature

Uveitis is the inflammation affecting one or more of the three parts of the eye that make

up Uvea: Iris, Ciliary Body and Choroid. Uveitis is broadly classified into Anterior,

Intermediate, Posterior and Pan Uveitis based on the anatomical involvement of the eye.

Uveitis can also involve adjacent structures like Sclera, Cornea, Retina, Vasculature and Optic

nerve head. Uveitis includes a large spectrum of inflammatory diseases that can occur either as

a co-manifestation of a non-infectious/autoimmune disorder, infection, malignancy or as a side

effect of medications and toxins, or an idiopathic ocular inflammation (no known cause).

Worldwide this disease is a sight threatening condition causing either transient or permanent

visual impairment and ocular complications that may or may not respond to therapy. A delay

in diagnosis and referral increases the risk that uveitis will result in irreversible damage to

various ocular structures. (20)

Epidemiology

Worldwide uveitis has reported annual incidence between 17 and 52.4 per 1,00,000

person years. The prevalence of uveitis is between 38 and 370 cases per 100,000 population, It

is particularly prevalent in younger people; the mean age of uveitis patients at the onset of the

disease is less than 40 years of age(21–23). Uveitis is the fourth most common cause of

blindness among the working-age population in the developed world, it accounts for about 25%

of blindness in developing countries. (1,2)

Darrell et al. in 1962 demonstrated that only 14% of uveitis patients were considered

elderly (>60 years of age). (22) However, this trend is changing especially in the developed

countries in the last two decades.

 Marwan R. et al in a systematic review found that the proportion of elderly (aging 60

years or more) among the uveitis patients is more in the developed countries (18.6%) compared

to the developing countries (7.2%). (25)

Idiopathic anterior uveitis is the most common form of uveitis in the community. (26)

Infectious causes are common (30-60%) in the developing countries. Herpes and

toxoplasmosis are the leading infectious causes of uveitis. Non-infectious uveitic conditions

are generally more common in the developed world but the prevalence of infectious etiologies,

including tuberculosis and syphilis are on the rise.

The Pacific ocular inflammation study (27) showed that there was no difference in the

incidence rate of uveitis between males and females. However, this study found that there was

a higher prevalence of uveitis in females. The incidence and prevalence of uveitis were also

found to increase with age.In a population study done in South India by Rathinam, et al

(28)from 1995-1997, the prevalence of uveitis was found to be 450/100,000 and age-adjusted

prevalence was 470/100,000. In this study, uveitis was found to be more common in males

compared to females. (28)

In a systematic literature review published in 2009 from Australia, (29) twenty two

published studies on the epidemiology and etiological patterns of uveitis, from Australia, North

and South America, Europe, Asia and Africa were analysed. They found that there were

similarities as well as differences in the pattern of uveitis found in different geographic regions

of the world, influenced by the geographical, environmental and genetic patterns. Anterior

uveitis was found to be the commonest in Europe, Australia and most parts of the United States,

followed by posterior uveitis. Idiopathic anterior uveitis was the commonest type of anterior

uveitis, followed by uveitis associated with seronegative spondyloarthropathy, Fuchs

heterochromic iridocyclitis, herpetic keratouveitis and sarcoidosis. Couto, et al (30) from

Argentina and Kotake, et al (31) found that panuveitis was more prevalent in Argentina and

Japan. Idiopathic uveitis was the commonest type of panuveitis in Asian countries like Japan,

Korea and Taiwan, followed by Behcet disease. (29)

In a study done in a tertiary referral centre in south India, Biswas, et al (6) documented

all new uveitis cases visiting their clinic over a two year period. Uveitis accounted for 1.5% of

all new cases in their department. Out of 1273 uveitis cases, anterior uveitis (39.28%) was the

commonest, followed by posterior uveitis (28.75%). Men (62.21%) were affected more than

women. People in their forties (23.57%) constituted the commonest age group at presentation.

Idiopathic, HLA-B27 negative cases accounted for most of the anterior uveitis (59.31%).

In a retrospective chart analysis done in a uveitis clinic in a tertiary referral centre in

North India, (5) the records of 1233 uveitis patients who were treated at the hospital from 1996-

2001, were analysed. Of the 1233 patients, 641 (51.98%) were males and 592 (48.01%) were

females. The mean age was 34.4 years (range 1.5 to 75 years). Among the different types of

uveitis, anterior uveitis was seen in 607 patients (49.23%), followed by posterior uveitis in 247

patients (20.23%), intermediate uveitis in 198 patients (16.06%) and panuveitis in 181 patients

(14.68%).

Specific etiology was found in 602 patients only. The commonest specific etiology of

uveitis in this study was infection, accounting for 29.73% (179 patients). Among the infective

cases, tuberculosis was the commonest (125 patients, 69.8%), followed by toxoplasmosis (21

patients, 11.7%). Non-infectious etiology of uveitis was found in 423 patients (10.06%), of

which ankylosing spondylitis was the commonest cause (80 patients, 18.9%), followed by

serpiginous choroidopathy (62 patients, 14.65%).

Classification and Nomenclature

The most commonly used classification of uveitis is devised by SUN Working Group

in 2005(Standardization of uveitis nomenclature). It is based on anatomic location of

inflammation(Table 1), duration and activity For the purpose of research, the SUN working

group (2005) has standardized definitions, grading of inflammation (Table 3 & 4) and outcome

measures.(2) More recently in 2008,the International Uveitis Study Group(IUSG) designed a

simplified classification of uveitis based on etiological criteria (Table 2).(3)

Table 1: The SUN Working Group Anatomic Classification of Uveitis(2)

Type Primary site of inflammation Includes

Anterior Anterior chamber Iritis,

uveitis Iridocyclitis,

Anterior cyclitis

Intermediate Vitreous Pars planitis

uveitis Posterior cyclitis

Hyalitis

Posterior Retina or choroid Focal, multifocal, or diffuse

uveitis choroiditis

Chorioretinitis, Retinochoroiditis

Retinitis Neuroretinitis

Panuveitis Anterior chamber, Vitreous

and Retina or Choroid

Table 2: International Uveitis Study Group (IUSG) 2008 (3)

Infectious Bacterial Viral Fungal Parasitic Others

Non- Known systemic association Not known systemic association

infectious

Masquerade Neoplastic Non-neoplastic

Table 3: The SUN Working Group grading scheme for anterior chamber cells

Grade Cells in Field(1mm x 1mm slit beam)

0 <1

0.5 1-5

1 6-15

2 16-25

3 26-50

4 >50
Table 4: The SUN Working Group grading scheme for anterior chamber flare

Grade Description

0 None

1 Faint

2 Moderate (iris and lens details clear)

3 Marked (iris and lens details hazy)

Duration and types of Uveitis (2)

Active uveitis is characterised by circumcorneal congestion, fresh keratic precipitates, grade

0.5+ or more cells in anterior chamber, (2) hypopyon, posterior synechiae, peripheral anterior

synechiae on gonioscopy, vitreous haze and cells and active inflammatory lesions in the retina

or choroid.

Features of inactive uveitis include absence of circumcorneal congestion, pigmented keratic

precipitates, grade 0 cells in anterior chamber,(1) pigments on anterior lens capsule, posterior

synechiae, peripheral anterior synechiae on gonioscopy, and chorioretinal scars suggestive of

old inflammatory lesions in the retina or choroid.

Onset of uveitis can be described as sudden or insidious.

Duration of uveitis is either limited, if it is 3 months or less in duration or persistent, if it is

greater than 3 months in duration.

Course of uveitis
Acute uveitis is defined as an episode of uveitis characterised by sudden onset and limited

(less than or equal to three months) duration.

Recurrent uveitis is repeated episodes of uveitis separated by periods of inactivity with

duration more than or equal to three months without treatment.

Persistent uveitis with relapse in less than three months after discontinuing treatment is termed

as chronic uveitis

Remission- inactive disease for at least 3 months after discontinuation of treatment.

Resistant to steroids - if there is no clinical improvement despite 2 weeks of treatment with

maximal dose.

Resistant to immunosuppressives - if there is no clinical improvement despite 3 months of

treatment.

Inactive anterior uveitis is defined as rare anterior chamber cells or less.

Improvement is defined as a 2 step decrease in the level of inflammation or a decrease to

‘inactive’.

Worsening is defined as a 2 step increase in the level of inflammation or an increase to

maximum grade.

Successful corticosteroid sparing is a reduction in the dose of prednisone to 10 mg per day

or less while maintaining inactive uveitis.

Granulomatous uveitis is characterised by large mutton fat keratic precipitates whereas

Non-granulomatous uveitis consists of small keratic precipitates.

Types of uveitis based on anatomic classification (2)

Anterior Uveitis

Definition: Anterior uveitis is the inflammation of the anterior chamber of the eye. It consists

of iritis (inflammation of the iris), anterior cyclitis (inflammation of the anterior part of the

ciliary body). (2)

Prevalence: Anterior uveitis is the most common type of uveitis, which accounts for 50-92%

of all uveitis cases in the West, and 28-50% in the Asian countries.(29) In a retrospective study

from South India done over a two year period, out of 1273 cases of uveitis, anterior uveitis was

the most common type, accounting for 500 cases (39.28%).(6)In a similar study done in North

India, anterior uveitis was found in 607 cases, accounting for 49.23% of the total number of

uveitis cases seen over a four year period.(5) In a retrospective study done on 308 uveitic

patients in North Eastern India, anterior uveitis was again found to be the most common type

(47.07%), followed by posterior(29.87%), intermediate(12.98%) and panuveitis (10.06%).

Males outnumbered females in this study. The mean age at presentation was 32.5 years in males

and 30.8 years in females, with a range of 2 to 61 years. (32)

Clinical Features

Symptoms: Patients present with redness, pain, watering, photophobia and blurred vision.

Some patients may be asymptomatic.

Signs: There may be circumcorneal congestion, keratic precipitates, pupillary miosis, posterior

synechiae and dilated iris vessels. The major indicators of anterior uveitis are the presence of

cells and flare in the anterior chamber. Gonioscopy may show peripheral anterior synechiae.

Depending on the duration of the inflammation, intra-ocular pressure may be normal, high or
low. In the acute phase, intra-ocular pressure may be reduced due to ciliary shut down caused

by the inflammatory process. Chronic uveitis with ciliary body atrophy may also present with

low intra-ocular pressure and the eye may become pthisical. Uveitic eyes may have increased

intra-ocular pressure due to trabeculitis, peripheral anterior synechiae or secondary steroid

response. (33)

Complications: Complications of anterior uveitis include cataract, glaucoma and cystoid

macular edema. HLA-B27 positive patients tend to have more recurrent and severe episodes of

anterior uveitis, and hence have been reported to have higher rate of complications.

Etiology: Idiopathic anterior uveitis is the most common type, accounting for 38-88% of

anterior uveitis.(19) The specific causes of anterior uveitis include seronegative

spondyloarthropathies (ankylosing spondylitis, reactive arthritis, inflammatory bowel disease,

psoriatic arthritis, undifferentiated arthritis), isolated HLA-B27 associated uveitis, Behcet

disease, Fuchs heterochromic iridocyclitis, sarcoidosis, syphilis and Tuberculosis.

Intermediate Uveitis

Definition: The term ‘intermediate uveitis’ is used for the type of uveitis in which the vitreous

is the major site of inflammation.

The SUN working group’s international workshop (2005) states that the diagnosis of

intermediate uveitis should not be changed with peripheral vascular sheathing and macular

edema.

Pars planitis: is an idiopathic Intermediate uveitis, when there is snowbank or snowball

formation, in the absence of an associated infection or systemic disease. (2)

Prevalence: In the West, intermediate uveitis has been reported in 1.4-22% of uveitis

patients.(2,7) Studies from India have found intermediate uveitis in 9.5-17.4% of uveitis

patients.(34) The prevalence is estimated to be 5.9/100,000 and incidence, 1.4/100,000.(6) The

prevalence of active intermediate uveitis was 0.25% in a South Indian study.(28) Though the

disease affects patients in all age groups, intermediate uveitis is mostly seen in the third and

fourth decade.(3,5,31)

Clinical Features

Symptoms: Patients with intermediate uveitis present with diminished vision and floaters,

Onset of intermediate uveitis is generally insidious. There is usually no pain, photophobia or

redness. (35)

Signs: The anterior chamber may be quiet or there may be minimal cells and flare, or

even a few keratic precipitates. The characteristic feature of active intermediate uveitis is

vitreous haze. Vitreous snowballs are yellow-white inflammatory aggregates, and are found in

mid vitreous and inferior periphery. Snowbanks are exudates on the pars plana. When present,

they are usually found inferiorly, but may extend to 360 degrees of the retinal periphery. Snow

banking is associated with a more severe form of disease, and hence warrants aggressive

therapy. Retinal changes include sheathing of peripheral veins, cystoid macular edema and

neovascularization. (35)

Complications: Complications of intermediate uveitis are mainly due to its chronicity, and can

lead to blindness. Chronic intermediate uveitis may lead to glaucoma, cataract, retinal

complications like macular edema and epiretinal membrane formation. (36)

Etiology: Chang et al (29) in their systemic review found that 60-100% of intermediate uveitis

found around the world was idiopathic. Specific diseases associated with intermediate uveitis

are sarcoidosis, multiple sclerosis and Lyme disease. (35)

Posterior uveitis

Definition: The primary site of inflammation is the retina or choroid. It includes focal,

multifocal or diffuse choroiditis, retinitis, retinochoroiditis, chorioretinitis and

neuroretinitis.(2)

Prevalence: Posterior uveitis accounts for 10.3-38.4% of the total number of uveitis cases. (6,

37, 38)

Clinical features

Symptoms: Posterior uveitis may be insidious in onset, or have an acute presentation with

floaters and blurred vision.

Signs: Posterior uveitis may present with vitritis, choroiditis, retinitis or retinovasculitis.

Complications: Decrease in visual acuity may occur due to different causes like the proximity

to macula, sequelae of choroidal or retinal neovascularisation, epiretinal membrane formation

or cystoid macular edema. (39)

Etiology: Idiopathic uveitis comprised 3-78% of posterior uveitis in the systemic review by

Chang ,et al.(29) The specific etiology of posterior uveitis are infective causes like

toxoplasmosis, toxocariasis, tuberculosis, syphilis, bartonellosis, viral infections like Herpes

simplex, Varicella zoster, Cytomegalovirus and HIV. The non-infectious types of posterior

uveitis include the white dot syndromes like Acute posterior multifocal placoid pigment

epitheliopathy(APMPPE), Multiple evanescent white dot syndrome(MEWDS), Geographic

helicoid peripapillary choroidopathy(GHPC), Multifocal choroiditis(MFC), Punctate inner

choroidopathy(PIC), Birdshot choroidopathy, Subretinal fibrosis and uveitis syndrome(SFU),

Diffuse unilateral subacute neuroretinitis (DUSN) and Retinal pigment epithelitis (Krill’s

disease. (40)

Panuveitis

Definition: The term ‘panuveitis’ refers to uveitis in which there is no predominant site of

inflammation, but inflammation is seen in the anterior chamber, vitreous and retina and or

choroid. (2)

Prevalence: Panuveitis accounts for 14.5-36.2% of the total number of uveitis patients in

various studies. (6, 37, 38)

Clinical features

Symptoms: Patients present with pain, blurring of vision, floaters and redness of the eye.

Signs: Severe anterior chamber reaction, vitritis, choroiditis, retinitis, retinal vasculitis and

optic disc edema may be seen in panuveitis.

Complications: Panuveitis is found to have the worst outcome among all types of uveitis.

Panuveitis can also lead to retinal ischemia, neovascularisation, secondary optic atrophy,

epiretinal membrane, complicated cataract and uveitic glaucoma. (39)

Etiology: 3-92% of pan uveitis seen world-wide is idiopathic. (29) 51.3% of pan uveitis seen

in India by Biswas, et al (6) was idiopathic. Vogt-Koyanagi-Harada(VKH) disease(21.08%)

and sarcoidosis(11.35%) have been reported to be the most common specific causes of

panuveitis in India.(34) Das et al(41) have reported that the most common etiology of pan

uveitis in North-Eastern India was VKH disease(45.06%), followed by sarcoidosis(29.03%).

Other reported specific causes are sarcoidosis, syphilis, Behcet disease and sympathetic

ophthalmia.

Uveitis due to infection

Tubercular Uveitis

Tuberculosis is a major cause of uveitis in underdeveloped countries. It is mostly seen

in young adults though no age is immune. It is seen slightly more among women than men. It

is a unilateral disease but bilaterality is seen in miliary tuberculosis. It can present as acute,

chronic or recurrent uveitis although chronic is more common. Less than 1% patients with

pulmonary or extrapulmonary tuberculosis suffer from uveal tuberculosis but the incidence of

frank tuberculosis in patients with uveal tuberculosis is rare. Iridocyclitis that does not respond

well to local steroids and cycloplegics without joint involvement, the first infective condition

that should arouse suspicion is tuberculosis.

Ocular manifestations may be caused by an active infection that invades the eye or by

a delayed type IV hypersensitivity immunological reaction to various antigenic components of

the mycobacteria, in the absence of the infectious agent. Most patients with ocular TB have no

history of pulmonary or systemic disease. The clinical manifestations of ocular TB vary

depending on mycobacterial virulence, host resistance to mycobacterium and the degree of

tissue hypersensitivity to mycobacterial antigens. Tuberculosis is primarily an airborne

(droplet) infection. The infection causes disease within the first few years after exposure in

about 5% of patients. In another 5% the disease may develop several years later as a result of
altered host immunity. About 90% of infected persons never develop any clinical disease and

remains asymptomatic throughout their lifetime. A positive PPD test can identify these infected

patients, (42–44)

In India, where pulmonary tuberculosis is endemic, Incidence of TB as a cause among

patients presenting with uveitis is variable(0.6%-10.1%).(5,6) In a study involving 1,005

patients with active pulmonary and extrapulmonary tuberculosis from South India, Biswas et

al reported an ocular morbidity of 1.39%. (7) .In 2003, study done at a tertiary referral centre

in north India, Singh et al found that out of 179 patients with infectious aetiological diagnosis,

the commonest was tuberculosis (125 patients; 69.8%). Tuberculosis was also the most

common cause of panuveitis (26.0% of panuveitis patients). (5) The increasing application of

PCR from intraocular fluids to diagnose tuberculous uveitis has led to an increase number of

patients being labelled with tubercular etiology. (45) The most common clinical presentations

are posterior uveitis, followed by anterior uveitis, pan uveitis, and intermediate uveitis.

Uveitis in Leprosy

The disease is caused by Mycobacterium leprae. About 60% of patients with leprosy

develop ocular manifestation and half of them develop uveitis, mostly bilateral. It takes 10-15

years for ocular involvement to be evident. It affects both the sexes equally in all ages.

Incidence in children is less. Uveitis is more common in lepromatous leprosy. The anterior

segment is more commonly and severely affected than the posterior segment. It can be acute

exudative iridocyclitis, chronic iridocyclitis or solitary leproma. The acute exudative

iridocyclitis is seen as part of type II lepra reaction. Leprotic pearls is seen in chronic

iridocyclitis. These are small white nodules spread all over the iris and sometimes on posterior

synechiae.
Syphilitic Uveitis

The disease is caused by Treponema pallidum. Uveitis can occur in both congenital and

acquired syphilis. The incidence of syphilitic uveitis is now increasing again in view of the

HIV epidemic. In congenital syphilis, uveitis can be either associated with interstitial keratitis

where the disease starts in the uvea and spreads to the cornea or without the same. It can be

associated with diffuse chorioretinitis that leaves a fundus picture of pepper and salt appearance

without night blindness. Uveitis in acquired syphilis is seen in late secondary stage or in tertiary

stage, between 3-12 months after primary infection. Anterior segment is more commonly

involved than the posterior segment. The lesions are predominantly granulomatous. The

iridocyclitis can be acute or gummatous iridocyclitis. The disease does not respond to

cycloplegic and steroids. (46–49)

Viral Uveitis

Common viruses that cause uveitis are herpes simplex, herpes zoster, cytomegalovirus,

Epstein-Barr and HIV. It is more common in immunocompromised patients. Herpes simplex

is the commonest virus that causes uveitis.

Herpes Simplex Uveitis

Herpes simplex uveitis is mostly recurrent, never primary. The duration of latent period

may vary from months to years. Keratouveitis is more commonly seen than iridocyclitis with

herpes simplex infection. The herpetic iridocyclitis is an acute, painful condition with

diminished vision. There is marked circumciliary congestion with a heavy aqueous flare,

hyphaema and large KPs. (50)

Cytomegalovirus Uveitis
 CMV infection is seen only in posterior uvea as either chorioretinitis or retinitis. About

80% of adults over 35 years of age are seropositive for CMV infection that they had suffered

either as congenital or as acquired asymptomatic disease. 1% of live births may have systemic

CMV infection, out of which only one tenth will show evidence of the disease in infancy and

childhood. About 50% of them will develop symptomatic ocular lesion, sensory neural

deafness and mental retardation. Acquired CMV infection occurs in 30% patients with AIDS,

mostly as retinitis. The lesions are bilateral, cotton wool spots, superficial haemorrhages with

periphlebitis, venous sheathing and vitreous haze. CMV retinitis may be the first presenting

sign of AIDS in a patient who has CD4 cell count less than 100 millimetre cube.

HIV manifestations

Posterior segment lesions are more common in AIDS than anterior segment

manifestations and can lead to severe ocular morbidity, often irreversible.

Cytomegalovirus is the most common infectious agent affecting the posterior segment.

It is seen in 15-40% of the patients. If the infection does not involve the posterior pole, patient

may be asymptomatic or present with decreased vision. Symptom of floaters could be an early

indication of the disease, although vitritis occurs occasionally only. Well established CMV

retinitis is easily recognised by ‘pizza pie’ appearance. 6% of cases with CMV retinitis can

have frosted branch angitis. Retinal detachment is seen in healed stage in 30% of cases. The

infection can occur 3-5 years after the diagnosis of AIDS and usually develops when CD4

counts are less than 50 cells per millimetre cube.

Uveitis can be the first presentation of systemic syphilis both in immunocompetent and

immunocompromised individuals on HAART. Syphilis presents with vitritis or panuveitis.

Ocular syphilis can cause significant inflammation inspite of low CD4 counts. Diagnosis can

be challenging in some (38%) HIV positive patients but remains sero-negative despite active

syphilitic disease. 85% HIV patients with ophthalmic syphilis have coexisting neurosyphilis.

Isolated episcleritis and scleritis are uncommon but are usually features of secondary and late

syphilis.

Cryptococcus neoformans is the most common fungal infective agent in AIDS. It

causes chronic chorioretinitis.

Candidial endophthalmitis is uncommon and is usually present in either highly drug

abusers or having indwelling catheters.

Ocular tuberculosis can present as anterior uveitis, choroiditis, choroidal granulomas,

chorioretinitis and panuveitis. Choroidal tuberculosis is common in HIV infection and it has

no correlation with CD4 counts. (39) Ocular TB has an aggressive course in AIDS patients

which might not resolve with ATT even when there is improvement in systemic TB. Initiation

of HAART before anti-TB therapy can lead to florid inflammation and paradoxical worsening

due to immune reconstitution inflammatory syndrome (IRIS). Regular ocular screening of

ocular TB is imperative in all cases of HIV in India inspite of good CD4 counts and regular

HAART.

Toxoplasma retinochoroiditis – Toxoplasma gondii protozoan affects 10% of patients

with AIDS but accounts for only 1% of AIDS related retinal infection. It is usually caused by

newly acquired infection and can cause progressive intraocular infection, panophthalmitis and

orbital cellulitis in AIDS. CNS lesions are seen in 25-50% patients with ocular toxoplasmosis.
Parasitic Uveitis

Toxoplasmosis

Toxoplasmosis is caused by Toxoplasmosis gondii, a protozoan. It is estimated to infest

10% of adults in northern temperate countries and more than half of adults in tropical countries.

A critical mode of human infection is transplacental hematogenous spread to the fetus in a

pregnant woman with active toxoplasmosis. In congenital toxoplasmosis, retinochoroiditis

occurs in over 75%, leaving scars. Acquired toxoplasmosis in immunocompetent adults is

subclinical in 80-90%. Toxoplasmosis constitutes 20-60% of all posterior uveitis. Reactivation

at previously inactive cyst-containing scars is he rule in the immunocompetent. ‘Spill over’

anterior uveitis may be granulomatous and resembles Fuchs uveitis syndrome with elevated

IOP. Vitritis may be severe and impair fundus visualization. White retinal inflammatory nidus

is viewed as ‘Headlight in the fog’. Vasculitis is more commonly venous. Toxoplasmosis

causes permanently reduced vision in 25% of eyes. Toxoplasma IgG antibodies are detectable

in the serum within 1-2 weeks of infection. PCR testing of intraocular fluid is variably sensitive

(16-67%) but highly specific. Ocular fluid antibody assessment by Goldmann-Witmer

Toxocariasis

Toxocariasis is caused by an intestinal ascarid (roundworm) Toxocara canis or

Toxocara cati. Ocular toxocariasis is associated with a lower parasitic load. It is typically

unilateral and in two-thirds causes some degree of permanent visual impairment.

Non Infectious Uveitis with known systemic association

HLA-B27 Associated Uveitis

HLA-B27 associated uveitis is described as acute, anterior, non-granulomatous,

recurrent and unilateral alternating. Males are affected between 1.5 to 2.5 times more than

females. HLA-B27 negative anterior uveitis does not show a gender difference. (13,33, 51–57)

HLA-B27 and the spectrum of associated inflammatory diseases have one of the

strongest Human Leukocyte Antigen and disease association known till date (21, 58–60). HLA-

B27 accounts for 18-32% of all cases of anterior uveitis in western countries. (37,38,61,62)

In a study done in North India, the HLA-B27 positivity rate was 56.2% among uveitis patients

and 3% in control samples.(63) The age of onset occurs between 20 and 40 years, whereas the

onset of HLA-B27 negative anterior uveitis usually occurs a decade later.(13,21,33,51,56,57)

HLA-B27 positive anterior uveitis can occur in isolation but they may go on to develop

spondyloarthropathy on later follow ups. Linssen, et al (57) found that after a follow up of 9

years, 12% of patients with isolated HLA-B27 positive anterior uveitis in their series,

subsequently developed spondyloarthropathy.

HLA-B27 associated anterior uveitis is known to cause severe sequelae like posterior

synechiae formation, cataract, cystoid macular edema, glaucoma and worse visual acuity

compared to other forms of anterior uveitis Based on studies done in tertiary referral centres in

the West, over 65% suffered from ocular complications of uveitis(57,64) Most common ocular

complications where Posterior synechiae (13 -91%) Cataract (7-28%)

Other complications include ocular hypertension, secondary glaucoma and the

development of chronic anterior uveitis. (13,33,51,56,57,64) The complications related to

HLA-B27 are usually confined to the anterior segment of the eye. However, posterior segment
complications (17-25%) are also reported. Around (6-13%) patients with HLA-B27 can

develop Cystoid macular edema (34,51,65) papilitis and severe vitritis have also reported. (64)

Posterior segment complications may be sight threatening, thus requiring aggressive

medical and surgical management. In the series by Rodriguez, et al (34) 32% of the HLA-B27

associated anterior uveitis patients with posterior segment complications required systemic

immunosuppressive therapy and in the series by Bayen, et al (66) 17% of the patients required

pars plana vitrectomy for control of inflammation and preservation of vision.

HLA type Associated disease

HLA-B27 Recurrent acute anterior uveitis

HLA-A29 Birdshot retinochoroidopathy

HLA-B51 and HLA-B5 Behcet syndrome

HLA-B7 and HLA-DR2 Ocular histoplasmosis syndrome

HLA-DR4 Sympathetic ophthalmitis

HLA-DR4 Vogt-Koyanagi-Harada syndrome

Ankylosing Spondylitis

Ankylosing spondylitis is a chronic inflammatory arthritis that primarily affects the

spine and sacroiliac joints. Ocular involvement occurs in 25% of patients with ankylosing

spondylitis. (60) Anterior uveitis is the most common manifestation. It is typically unilateral,

but can be bilateral or alternating. Both eyes are involved in 80% patients, but they are rarely

inflamed simultaneously Recurrence of the ocular inflammation may occur as frequently as

every 2-3 weeks. (67) Anterior uveitis associated with ankylosing spondylitis usually presents

as a unilateral, acute iritis with pain, photophobia and redness.

Reactive Arthritis

Reactive arthritis may occur after dysentery caused by Gram-negative bacteria like

Shigella, Salmonella, Campylobacter. (67) Most patients have signs 2-4 weeks after the onset

of dysentery, but the full syndrome may take years to develop. (68) It has a male preponderance

and onset of symptoms is generally between 18-40 years. Conjunctivitis is the most common

ocular finding in patients with reactive arthritis, and occurs in 30-60% patients. It is usually

mild and bilateral. Anterior uveitis occurs in 3-12% patients. It is usually non-granulomatous

and may have mild cellular reaction, hypopyon, peripheral anterior synechiae and trabeculitis.

Patients can also present with keratitis, scleritis and episcleritis. Scleritis is usually in the form

of diffuse anterior scleritis and never progresses to necrotizing scleritis.

Psoriatic Arthritis (69)

Uveitis occurs predominantly in patients who develop arthropathy. About 20% of

patients with psoriasis develop psoriatic arthropathy and about 20% of these patients develop

uveitis, sacroiliitis and ascending spine disease (F76). Anterior uveitis is similar to that found

in ankylosing spondylitis and reactive arthritis.

Undifferentiated Spondyloarthritis

A diagnosis of undifferentiated spondyloarthropathy is made when the patient fulfils

the criteria for spondyloarthropathy without evidence suggesting a more specific disorder. The

disease can either remain the same for many years or can progress into well-defined subsets of

spondyloarthropathy. Majority of the patients (59-68%) may progress to ankylosing spondylitis

within 3-11 years. (70–72)

Vogt-Koyanagi-Harada (VKH) Disease

VKH is an idiopathic multisystem autoimmune disease with Granulomatous

inflammation of melanocyte containing tissues such as the skin, uvea, ear and meninges. VKH

predominantly affects Hispanic, Japanese and pigmented individuals. It is associated with

HLA-DR1 and HLA-DR4.

Vogt-Koyanagi-Harada disease uveitis is a severe bilateral diffuse non-necrotizing

granulomatous intraocular inflammation (posterior or panuveitis) associated with serous retinal

detachments, disk edema, and vitritis, with eventual development of a sunset glow fundus, is

an autoimmune inflammatory condition mediated by CD4+ T cells that target melanocytes in

individuals susceptible to the disease.

 The trigger that induces altered tolerance to melanocytes is still not known. The

tyrosinase peptide antigen is the target of autoimmunity by T lymphocytes on recognition of

HLA DRB1*0405 expression by the melanocytes. Infections may play a role in initiating the

autoimmune process. The lack of prior inciting trauma differentiates it from sympathetic

ophthalmia. (73–76)

Vogt-Koyanagi-Harada disease presents clinically in 4 different phases: prodromal,

uveitic, convalescent, and recurrent, with extraocular manifestations including headache,

meningismus, tinnitus, hearing loss, poliosis, and vitiligo, to varying degrees. (77,78)

The mainstay of treatment has been corticosteroids and, more recently, steroid-sparing

immunomodulators for long-term control.

Epidemiology and geographic distribution

The prevalence of VKH varies in different populations in the world, being more

common in Asia, Latin America, and the Middle East with prevalence rates ranging from 4.4%

to 21%.(79) VKH is not common in the United States and makes up only about 3% to 4% of

referrals to tertiary care centres.(80)

The patients diagnosed with VKH were predominantly female (69-77%), Hispanic

(54%-78%) and a mean age of 33.5 years (range 7 to78 years) VKH appears to be more

common in pigmented individuals and is relatively rare in whites.(79,81,82) VKH was the most

common cause of panuveitis in India, with a prevalence of 21.08%. (6)

Vogt-Koyanagi-Harada disease (VKH) in children

Martin et al studied new cases of uveitis in South India and noted 1.2% of patients with

a diagnosis of VKH of which 8.2% were children. Age of presentation was 8 to 16 years, and
median age was 13.5 years. Most children ultimately did well, with a final vision of better than

6/12 in 75%.

Hamade IH et al, in a study done in Saudi Arabia, the prevalence of VKH in children

younger than 16 years of age was as high as 16%, behind acute anterior nongranulomatous

uveitis and intermediate uveitis. This study found that 54% of children with VKH had vision

better than or equal to 6/12. But had the highest rate of complications; that is, glaucoma in

50%, papilitis in 53%, epiretinal membrane in 16%, and retinal detachment in 4%. Children

were at risk of amblyopia secondary to VKH. (83,84) Patients over age 60 years (age range 60-

86 years) were found to have a lower prevalence of VKH compared to younger patients.

Patients above age 65 years had a higher incidence of optic disk hyperemia, choroidal

detachment, and cataract compared to younger patients. They were also more likely to need a

higher dose of corticosteroids and often maintained good vision despite low-grade smoldering

inflammation.(85,86)

The prodromal phase may present a viral infection and lasts for few days to few weeks.

Clinical manifestations are predominantly extraocular including headache (82%),

meningismus (55%), fever (18%), nausea (9%), vertigo (9%), orbital pain, and auditory

disturbances. Cerebrospinal fluid may show pleocytosis in more than 80% of patients. (74)

Acute uveitic phase sudden onset, bilateral granulomatous uveitis is seen in upto 70% of

patients presents with pockets of subretinal fluid, hyperemia of the optic nerve head and

choroidal thickening causing blurring of vision and conjunctival injection. Anterior segment

shows granulomatous reaction with the presence of mutton fat keratic precipitates. Forster et

al reported that an increase in IOP is seen in up to 54% of patients and 38% of patients requiring

medical or surgical intervention.

In Convalescent phase Depigmentation of the choroid, sunset glow fundus, vitiligo, and

poliosis occurs. , progression occurs in 28%–62% of patients who present with acute VKHD.

Chronic recurrent phase is characterized by exacerbations of granulomatous anterior uveitis

that is usually resistant to systemic steroid therapy and with later involvement of the

choriocapillaris. It usually develops 6 to 9 months after initial presentation and is also marked

by complications- retinal pigment epithelium proliferation, subretinal fibrosis(6%), subretinal

neovascular membranes(3%-11%), posterior subcapsular cataract (15%-45%), posterior

synechiae (23.4%), band keratopathy(5.2%) open-angle glaucoma (27%-33%), and,

occasionally, angle-closure glaucoma. Forster et al found that recurrent inflammation was a

significant risk factor for poor visual outcome. (87–89)

Extraocular manifestations

Neurologic signs, occur more commonly during the prodromal phase. 18-50% of

patients have some form of sensory hearing loss, mostly at higher frequencies of 4, 6, and 8

kHz.154, Tinnitus is present in 42% of the patients. (90,91)

Integumentary changes associated with VKH develops in about 30% of patients.(92) It

occurs in the convalescent phase, when depigmentation of the choroid occurs, the eyebrows,

eyelashes, hair, and skin also lose pigment, resulting in poliosis and vitiligo.(93,94)

The initial treatment is with high-dose corticosteroids, then steroid-sparing therapy,

Despite early treatment, progression to the chronic recurrent phase may occur in up to 79% of

the total cases who presented with acute VKHD.4 VKH is a severe inflammatory disease, but

prompt and aggressive treatment, may lead to better visual outcomes 6/ 6-6/18 .(95–97)
Modified diagnostic criteria for Vogt-Koyanagi-Harada syndrome

1.Absence of a history of penetrating ocular trauma

2.Absence of other ocular entities

3.Bilateral uveitis

4.Neurological and auditory manifestations

5.Integumentary findings, not preceding onset of central nervous system or ocular disease

such as alopecia, poliosis and vitiligo.

Sympathetic Ophthalmitis

Sympathetic ophthalmitis is a bilateral granulomatous panuveitis occurring after

penetrating trauma. Less frequently the condition occurs following intraocular surgery.

Presentation in trauma induced cases is between 2 weeks and 3 months after initial injury in

65%. The incidence is probably 0.2%-0.5% after injury and 0.01% following intraocular

surgery.

Sarcoidosis

Sarcoidosis is a chronic multisystem disorder of unknown cause, manifesting with non-

caseating granulomatous inflammatory foci. It has an incidence rate of 6-10 per 1,00,000. It

affects lungs, lymph nodes, skin, liver, eye, heart and muscles. Ocular inflammation occurs in

25-70% of sarcoid patients depending on ethnicity. Granulomatous anterior uveitis is the most

common manifestation. Women are affected in 41% of cases in India.

The ACCESS study a multicentre study from 10 specialist centres across the United

States. Patients usually present between 20 and 40 years of age .Women were more likely to
be affected. In clinical manifestations; women tended more to ocular and neurological

involvement, whereas men had a higher risk of hypercalcemia. (98,99)

In United Kingdom, 5.9% of patients have an affected relative, (100) the relative risk

of sarcoidosis for family members in the United States is 4.7, siblings being at highest risk 5.8.

There are differences in disease prevalence between countries. In Japan this is as low as

3.7:100,000, compared to Finland where it is as high as 28.2:100,000. (101)

Sarcoid uveitis accounts for 3-10% of all cases of uveitis. The International workshop

on Ocular Sarcoidosis (IWOS), 2009 has set up the criteria for diagnosis of intraocular

sarcoidosis. (16)

Common clinical manifestations Systemic sarcoidosis dyspnoea, dry cough, or wheeze

(31-50%). bilateral hilar lymphadenopathy (79%-95%), skin involvement (25%) subclinical

hepatic involvement (75%), clinically significant Cardiac sarcoidosis (<5%), Neurological

involvement (5–26%) of these facial or optic neuropathies (50–75%), (15,102,103)

The onset of Ocular Sarcoidosis is usually insidious and has a chronic course patients

presents with blurred vision, floaters, redness, or discomfort, In biopsy proven cases ocular

features include granulomatous uveitis, iris nodules (52%).

Trabecular meshwork nodules and/or tent-shaped PAS (81%), Ocular hypertension

(34%), Snowball/string of pearls vitreous opacities (69%). Multiple peripheral chorioretinal

lesions active and/or atrophic(76%), Nodular and/or segmental periphlebitis (± candlewax

drippings) and/or retinal macroaneurysms (45%), Optic disc nodule(s)/granuloma(s) or solitary

choroidal nodule(6%), Bilaterality(98%).Common complications of chronic ocular sarcoidosis

are Cataract, Glaucoma, and Cystoid macular edema(16,17,67,104)

Behcet disease

Behcet disease is an idiopathic, multisystem syndrome characterised by recurrent

aphthous oral ulcers, genital ulceration and uveitis. Vasculitis is a key pathogenetic component

and may involve small, medium and large veins and arteries. It is strongly associated with

HLA-B51. The peak age of onset is the third decade. The International Study Group for Behcet

disease (ISGBD), 1990 established criteria for diagnosis of Behcet disease.

Ocular inflammation occurs in about 70% and tends to be more severe in men. Signs

are always bilateral eventually. Relapsing or remitting acute onset panuveitis with retinal

vasculitis and often spontaneous resolution even without treatment is the classical pattern of

eye involvement. Retinal vascular disease (vasculitis and occlusion) is the main cause of visual

impairment.

Childhood Uveitis

Non-infectious paediatric uveitis can lead to ocular complications and vision loss. It is

most often associated with juvenile idiopathic arthritis (JIA), but can be observed in other

autoimmune conditions like Behcet’s disease or sarcoidosis. Idiopathic uveitis is common as

JIA-associated uveitis (JIA-U). Anterior uveitis is the most common manifestation. Ocular

complications are reported in up to 50% of children. Moderate visual impairment affects 25-

40% children and severe visual impairment in upto 25% children. Angeles-Han reported that

the odds of developing juvenile idiopathic arthritis associated uveitis increases nine fold when

the subjects carried both HLA-DRB1*11 and *13. Association between autoimmune chronic

uveitis in children with the presence of NOD2/CARD15 gene was reported by Marrani, et al.
 Risk factors for sight-threatening complications in paediatric non-infectious uveitis

include short duration between arthritis and uveitis diagnoses, young age at uveitis onset, male

gender, uveitis diagnosed prior to arthritis, and the presence of vision loss or complications at

first ophthalmology exam. (105,106)

Juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common group of systemic diseases

causing uveitis in childhood. It has a prevalence of 10 per 100 000 persons. It occurs more in

girls (75–80%) and 70–90% of them are antinuclear antibodies positive JIA often has a severe

inflammatory course, and endangers vision.

Approximately 10–20% of children with JIA are at risk for uveitis. Those with positive

antinuclear antibodies (ANA), are young at arthritis diagnosis (≤ 6 years old), have

oligoarticular or polyarticular RF negative JIA, and early in their disease course (≤ 4 years) are

considered high risk. The intraocular inflammation is mostly diagnosed between the 4th and

6th years of life shortly before or within four to five months after arthritis onset, in some 75%

within a year, in 90% within four years, and in only 3–5% before or five or more years after

the onset of JIA. In 75% of children the inflammation affects both eyes, simultaneously or

within a few months of each other. Current screening guidelines recommend monitoring these

children every 3–4 months. (105,106)

The diversity of etiology in uveitis occurs due the influence of factors like geographic

location, regional differences, climatic changes, occupation and socioeconomic profile. (107)

Epidemiology of uveitis varies in different parts of our country. In a study from a referral centre

in south India, the prevalence of uveitis was reported to be 1.5% of new cases presenting to the
centre. Anterior uveitis was the most common diagnosis, males were more affected, age group

was 40 years and the commonest etiology among the anterior uveitis was idiopathic

inflammation.(6) In another study, leptospiral, tuberculous and herpetic disease was identified

in patients with uveitis as most common identifiable infectious etiology. Parasitic uveitis was

common in children less than 16 years of age and herpetic etiology the most common in those

above 60 years of age. (107) In another study from north India, anterior uveitis was the

commonest presentation, males were more affected. The 2 commonest infectious etiology

detected were tuberculosis followed by toxoplasmosis. Among the non-infectious causes

ankylosing spondylitis was the most common. (5)

In our study we would like to describe the anatomic, demographic and etiologic profile

of patients attending our uveitis clinic and compare it to the patterns of uveitis globally and

from different parts of our country.

Methodology

This was a hospital-based cross-sectional study. The study was conducted over a

twelve-month period, from November 2015 to November 2016, after receiving the approval

of the Institutional Review Board (IRB Min No. 10338).

Patients who attended the Uvea Clinic of the Department of Ophthalmology, Christian

Medical College, Vellore with a presumed or proven diagnosis of uveitis were included in the

study. A detailed history was elicited from all patients. Every patient in the study had a

complete ophthalmological evaluation to look for evidence of past or present uveitis. All

patients were examined using a slit lamp biomicroscope. The diagnosis of uveitis was made

based on established international diagnostic criteria. (3,16,36,74,108–110) The diagnosis of

uveitis was confirmed by consultant ophthalmologists in the department, with more than ten

years of experience in the specialty. Appropriate investigations were done when indicated

Systemic diagnosis were confirmed (or ruled out) through consultation with concerned

specialists (adult/paediatric Rheumatologist, adult/paediatric Infectious disease specialist).

Inclusion Criteria

Patients with diagnosis of anterior/ intermediate/ posterior/ pan uveitis and scleritis.

Exclusion criteria

1. Uveitis in the same eye within 3 months of trauma /surgery or any intraocular

procedures.

2. Any other disease which may be associated with or masquerade as uveitis.

Data collection

Data collection was done using a ‘Clinical research form’ (Appendix II). Clinical data,

investigations and treatment details were recorded. Form included details pertaining to

 Age, Gender, Occupation, Residence,

 Family history pertaining to systemic illness (Connective tissue/ autoimmune/

infection)

 Medical history of Systemic illness, Exposure to infection/animals

 Ocular history- Number of episodes, Laterality and ocular symptoms of Initial episode

of Uveitis

 Past treatment history, Compliance, Response to treatment, Treatment complications

 Chief compliant-(reason for visit), Laterality, ocular symptoms in the current visit

 Systemic evaluation

 Ocular examination: Visual acuity, complete clinical ocular examination using slit-

lamp biomicroscopy, indirect ophthalmoscopy and Intraocular pressure

 Relevant investigations

 Final Laterality and course, Anatomical diagnosis, Etiological diagnosis, Treatment

plan

Data was analysed with IBM Inc. SPSS version 21

Data pertaining to age, gender, location of uveitis, chronicity, and diagnosis were extracted

and analysed in accordance with the International Uveitis Study Group (IUSG)

recommendation,.(3)
Definitions

Anterior uveitis is defined as the inflammation of the anterior chamber of the eye. It consists

of iritis (inflammation of the iris), anterior cyclitis (inflammation of the anterior part of the

ciliary body). (2)

Intermediate uveitis is defined as inflammation with the vitreous as the major site of

inflammation. The presence of peripheral vascular sheathing and/ or macular edema will not

change the diagnosis of intermediate uveitis.

Posterior uveitis is defined as the inflammation where the primary site of inflammation is the

choroid or retina. It includes focal, multifocal or diffuse choroiditis, retinitis, retinochoroiditis,

chorioretinitis and neuroretinitis. (2)

Panuveitis is defined as the inflammation with no predominant site of inflammation but

involves anterior chamber, vitreous and choroid.

Definitions of uveitis according to the course of the disease

Acute uveitis is defined as an episode of uveitis characterised by sudden onset and limited

duration (less than or equal to three months).

Recurrent uveitis is defined as repeated episodes of uveitis separated by periods of inactivity

with duration more than or equal to three months without treatment.

Chronic uveitis is defined as persistent uveitis with relapse in less than three months after

discontinuing treatment.

Improvement in uveitis is defined as a two-step decrease in the level of inflammation or

disease became inactive.

Worsening in uveitis is defined as a two-step increase in the level of inflammation or an

increase to maximum grade.

Granulomatous uveitis is characterised by large mutton fat keratic precipitates.

Non-granulomatous uveitis is characterized by absence of large mutton fat keratic

precipitates. Small keratic precipitates are seen.

International Uveitis Study Group Etiological classification

Infectious uveitis:

 Bacterial

 Viral

 Fungal

 Parasitic

 Others

Non-infectious uveitis:

 Known systemic association

 Not known systemic association (Idiopathic uveitis)

Masquerade syndromes:

 Neoplastic

 Non-neoplastic
Revised Diagnostic criteria for Vogt-Koyanagi-Harada disease (International Committee

of Nomenclature, 2000) (111)

Complete Vogt-Koyanagi-Harada syndrome

I. No history of penetrating ocular trauma or surgery

II. No clinical or laboratory evidence of other ocular or systemic disease

Ill. Bilateral ocular disease (either A or B below must be met):

A. Early manifestations

1. Diffuse choroiditis as manifested by either:

a. Focal areas of subretinal fluid, or

b. Bullous serous subretinal detachments

2. With equivocal fundus findings, then both:

a. Fluorescein angiography showing focal delayed choroidal perfusion,

pinpoint leakage, large placoid areas of hyperfluorescence, pooling of

dye within subretinal fluid, and optic nerve staining

b. Ultrasonography showing diffuse choroidal thickening without

evidence of posterior scleritis

B. Late manifestations

1. History suggestive of findings from IIIA. and either both 2 and 3 below, or

multiple signs from 3

2. Ocular depigmentation

a. Sunset glow fundus, or b. Sugiura sign

3. Other ocular signs

a. Nummular chorioretinal depigmentation scars, or

b. RPE clumping and/or migration, or

c. Recurrent or chronic anterior uveitis

IV. Neurologic/auditory findings (may have resolved by time of examination):

A. Meningismus

B. Tinnitus

C. Cerebrospinal fluid pleocytosis

V. Integumentary findings (not preceding central nervous system or ocular disease)

A. Alopecia

B. Poliosis

C. Vitiligo

Incomplete Vogt-Koyanagi-Harada syndrome

Criteria I to III and either IV or V from above

Probable Vogt-Koyanagi-Harada syndrome

Criteria I to III from above must be present

Isolated ocular disease

International Criteria for the Diagnosis of Ocular Sarcoidosis: First International

Workshop on Ocular Sarcoidosis (IWOS) 2009 (16)

Intra ocular signs

(1) Mutton-fat keratic precipitates (KPs)/small granulomatous KPs and/or

Iris nodules (Koeppe/Busacca)

(2) Trabecular meshwork (TM) nodules and/or

Tent-shaped peripheral anterior synechiae (PAS)

(3) Vitreous opacities displaying snowballs/strings of pearls

(4) Multiple chorioretinal peripheral lesions (active and/or atrophic)

(5) Nodular and/or segmental peri-phlebitis (± candlewax drippings) and/or

Retinal macroaneurism in an inflamed eye

(6) Optic disc nodule(s)/granuloma(s) and/or

Solitary choroidal nodule

(7) Bilaterality

The laboratory investigations

(1) Negative tuberculin skin test in a BCG-vaccinated patient or

Negative in a patient having had a positive tuberculin skin test previously

(2) Elevated serum angiotensin converting enzyme (ACE) levels and/or

Elevated serum lysozyme

(3) Chest x-ray revealing bilateral hilar lymphadenopathy (BHL)

(4) Abnormal liver enzyme tests

(5) Chest CT scan in patients with a negative chest x-ray result

In inpatients whom other possible causes of uveitis had been excluded

TABLE-1 Level of certainty of having sarcoidosis

1 Definite ocular sarcoidosis Biopsy-supported diagnosis

With compatible uveitis

2 Presumed ocular sarcoidosis Biopsy not done but

Chest x-ray positive showing bilateral

hilar lymphadenopathy

With compatible uveitis

3 Probable ocular sarcoidosis Biopsy not done

Chest x-ray did not show bilateral hilar

lymphadenopathy

With at least 3 of the intraocular signs

and 2 positive laboratory tests

4 Possible ocular sarcoidosis Lung biopsy negative

But at least 4 of the intraocular signs and

2 positive laboratory investigations

TABLE -2 The International Criteria for Behcet’s Disease (ICBD) 2006 (112)

Clinical manifestations Score

I Oral aphthosis (OA) 1

II Genital aphthosis (GA). 2

III Skin manifestations, 1

Pseudofolliculitis (PF)

Erythema nodosum (EN)

IV Ocular manifestations 2
Anterior uveitis (AU)

Posterior uveitis (PU)

Retinal vasculitis (RV)

V Pathergy phenomenon (PP) 1

VI Vascular manifestations (VMs) 1

Superficial phlebitis

Deep vein thrombosis

Large vein thrombosis,

Arterial thrombosis

Aneurysm

A patient has to get three or more points to be diagnosed/classified as having

Behcets’ disease.

.
DIAGNOSTIC CRITERIA FOR INTRAOCULAR TUBERCULOSIS (110,113)

Clinical presentations with not associated with any other known clinical entity

1. Granulomatous anterior uveitis

2. Non-granulomatous anterior uveitis, not associated with any other known clinical entity

3. Intermediate uveitis, with/without healed/active focal lesions

4. Posterior uveitis, including subretinal abscess, choroidal/disc granuloma, multifocal

choroiditis, retinal periphlebitis and multifocal serpiginous choroiditis

5. Panuveitis

6. Rarely, scleritis (anterior and posterior), interstitial and disciform keratitis.

Diagnostic categories

Presumptive ocular TB

A patient with one of the clinical presentations and no association with any other known

clinical entity

Possible ocular TB (1, 2 and 3 together or 1 and 4)

1. At least one clinical sign suggestive of ocular TB and other aetiologies excluded

2. X-ray/CT chest not consistent with TB infection and no clinical evidence of extraocular

TB

3. At least one of the following:

a. Documented exposure to TB

b. Immunological evidence of TB infection

4. Molecular evidence of Mycobacterium tuberculosis infection.

.
Probable ocular TB/Clinically diagnosed ocular TB (1, 2 and 3 together)

1. At least one clinical sign suggestive of ocular TB and other aetiologies excluded

2. At least one of the following

a. Evidence of chest X-ray consistent with TB infection

b. Clinical evidence of extraocular TB

c. Microbiological confirmation from sputum or extraocular sites

3. At least one of the following:

a. Documented exposure to TB

b. Immunological evidence of TB infection

Bacteriologically confirmed ocular TB (1and 2 together)

1. At least one clinical sign of ocular TB

2. Confirmation of Mycobacterium tuberculosis from ocular fluids/tissues

a. Microbiological (smear/culture) or

b. Histopathological

Note: Extraocular TB disease is often absent in ocular TB patients, and patients do not usually

have systemic symptoms of fever and weight loss.

.
Vision assessment

Vision (Presenting distance visual acuity) was recorded with the use of Snellen’s chart

at six metres and was categorized according to the revised World Health Organization

Classification.

TABLE-3 Classification Visual impairment including blindness (binocular or

monocular).(114)

Category Presenting distance visual acuity

Worse than: Equal to or better than:

0 Mild or no visual impairment 6/18

1 Moderate visual impairment 6/18 6/60

2 Severe visual impairment 6/60 3/60

3 Blindness 3/60 1/60 or Counting fingers

(CF) at one metre

4 Blindness 1/60 or Counting Light perception

fingers (CF) at one

metre

5 Blindness No light perception

9 Undetermined or unspecified

.
TABLE -4 Visual impairment (binocular or monocular)
Worse than Equal to or better than Category

6/12 No visual impairment

6/12 6/18 Mild visual impairment

6/18 6/60 Moderate visual impairment

6/60 3/60 Severe visual impairment

3/60 Blindness
RESULTS

One hundred and four patients were recruited into our study from November 2015 to November

2016. The patients presented to us from different parts of the country. The majority of the

patients were from Tamil Nadu, especially Vellore.

Age distribution

The age of the patients in our study ranged from 3 to 87 years. The mean age was 38.75 years

(SD – 16.049 years).

Figure-1 Age Distribution

Gender Distribution

There were 51 males (49%) and 53 females (51%) in the study. The age range in boys/men

was 12-87 years and in girls/women was 3-66 years.

Figure-2 Gender Distribution

Distribution of Occupation

Of the 104 patients, 34 (32.7%) were housewives, 17 (16.3%) were students, 9 (8.7%) were

coolie workers, 8(7.7%) were farmers.

Figure-3 Distribution of Occupation

Figure-4 MAP OF INDIA with depicting the State of residence of the study patient

2
12

7
2
76

76 patients (73.2%) were from Tamil Nadu. Of these 66 patients (86.8%) were from Vellore.

12 patients (11.5%) were from West Bengal. 7 patients (6.8%) were from Andhra Pradesh
Only 4 patients were able to recollect a positive family history of any connective tissue

disorder.

FIGURE-5
FAMILY HISTORY OF CONNECTIVE TISSUE
DISORDER
Interstitial Lung disease

Unspecified connective tissue

with Uveitis
Psoriasis
1 1
Rheumatoid arthritis

1 1

Family History of Infectious disease

Three patients had family history of exposure to tuberculosis. Two of these patients had

systemic tuberculosis concurrent with the diagnosis of ocular disease.

Figure-6 pulmonary tuberculosis

 34 years old female with TB associated

bilateral recurrent multifocal choroiditis.

 46 years old female with TB associated

chronic bilateral anterior uveitis.

 24 years old male with idiopathic chronic

non-granulomatous unilateral anterior uveitis

MEDICAL HISTORY OF SYSTEMIC ILLNESS

Thirty two patients (31%) had history of systemic illness. Of these 32 patients, 17 patients

(53%) had inflammatory joint related pathology.

Figure-7 medical history of systemic illness

Figure -8 Associated systemic illness

Medical History of exposure to Infectious disease

Twenty six patients (25%) had proven systemic diagnosis of infectious disease. 7 patients

(6.7%) had tuberculosis, 5 patients (4.8%) had HIV.

Figure -9&10 medical history of exposure to Infectious disease

History of Exposure to Animals

Only 2 patients (1.9%) had history of exposure to animals.

Animal Final diagnosis

Idiopathic bilateral recurrent anterior

uveitis

DOG

Viral recurrent unilateral Uveitis

CAT

.
Ocular Injury

Eight patients (7.7%) had ocular injury

Figure -11 Ocular Injury

Four (3.8%) had closed globe injury, three (2.9%) had open globe injury and one (1%) had

chemical injury.

Figure -12 Type of injury

Of the eight patients with ocular injury, 4 had uveitis in the same eye while the other four had

injury to the opposite eye.

Figure -13 Laterality and Type of injury

Number of Episodes- Among the 104 patients, for 44 patients (42.3%) it was the first episode

of uveitis while 60 patients (57.8%) had more than one episode. Of the patients with first

episode, 21 (47.7%) were in 21-40 years age group and 11 (25%) were below the age of 20

Figure -14 Number of Episodes

More males presented with first episodes

Figure-15 & 16 Gender and Age Distribution among patients with 1st Episodes of uveitis
Laterality of Symptoms at Presentations

At the onset of Uveitis 57 patients (54.8%) had unilateral eye symptoms, 43 patients (41.3%)

had bilateral symptoms but at the current presentation 50% (n=52) had bilateral eye symptoms

at presentation. 49 patients (47.1%) had unilateral eye symptoms and 3 patients (2.9) had

alternating eye symptoms.

Figure -17 Laterality of symptoms in primary presentation

Figure -18 Laterality of symptoms in current presentation

Comparison of symptoms in Initial and Current episodes

Both at initial onset of uveitis and current presentations visual disturbance was the most

common symptom followed by redness, pain, photophobia and watering respectively. Eighty

one patients (77.9%) had visual disturbances at the first episode but this increased to 87.5%.

by the time they arrived at the clinic.

Figure -19 & 20 symptoms at presentations

.Table-( ) Symptoms At onset of uveitis n, (%) At current presentation

of uveitis n, (%)

Visual Disturbance 81 (77.9%) 91 (87.5%)

Redness 62 (59.6% 60 (57.7%)

Pain 48 (46.2%) 48 (46.2%)

Photophobia 31 (29.8%) 34 (32.7%)

Watering 19 (18.3%) 20 (19.2%)

Type of Visual Disturbances

Figure-21 Visual disturbances during primary episode

70
63
60

50

40

30
23
20

10 8 8

1 1
0
Decreased vision Diffuse blur Floaters Central Scotoma Peripheral No visual
Scotoma disturbance

Figure-22 Visual disturbances during current episode

80
72
70

60

50

40

30

20
11 13
10 8
1 1
0
Decreased Diffuse blur Floaters Central Peripheral No visual
vision Scotoma Scotoma disturbance

Decrease in vision was the commonest among visual disturbances both at onset (n=63, 60.6%)

and at current presentation (n=72, 69%). Next common symptoms were diffuse blurring of
vision and floaters.

Treatment History

Out of 104 twenty three patients (22.1%) had no prior treatment for uveitis at presentation (first

visit to eye care centre) Thirty nine patients (37.5%) were already on medication at the time of

current presentation. Of these 82% (n=32) had anterior uveitis.

Figure-23 Treatment history at presentation

45

40 39

35

30

25

20
20

15
11
10

5 3 3
2
1 1 1
0
Treatment Compliance

Eighty one patients were on prior treatment and 54 patients (67%) gave history of good

treatment compliance.

Figure-24 Treatment Compliance

Treatment response

Fifty patients (62%) showed improvement while 16 patients (20%) had no improvement of

symptoms on treatment. Five patients (6%) worsened while still on treatment and 12% patients

(n=10) worsened when they went off treatment. Of the 54 patients with good compliance, 82%

patients (n=46) showed improvement on treatment while 11% patients (n=6) had no

improvement and 3.7% (n=2) worsened on treatment.

Figure -25 Treatment response

Complications due to treatment were found in 37 patients (45.68%). Of these, 19 (51%)

developed cataract and 18 (49%) showed steroid response.

Figure -26 Treatment complications

Figure-27 Types of complications

PRESENTING DISTANCE VISUAL ACUITY (according to laterality of disease)

Forty two patients (40 %) had unilateral disease and 62 patients (60 %) had bilateral disease

Figure-28 Affected eye vision in unilateral disease

Figure-29 Better eye vision in bilateral disease

Visual impairment

Visual impairment was present in 33(31.73%) patients. Of these 33 patient, 24 patients had

bilateral disease

Figure-30 visual impairment

Figure-31 Visual impairment based on Presenting distance visual acuity

INTRAOCULAR PRESSURE

On presentation 51.9% (n=54) patients had IOP within normal range while 19% (n=20) had

low IOP and 14% (n=15) had high IOP. High IOP due to steroid response was found in 14%

(n=15) patients

Figure-32 Intraocular pressure

. KERATIC PRECIPITATES 75 patients had evidence of KP of these 14(19%) had

granulomatous KPs and 61 (81%) had non-granulomatous type

Figure-33 Type of Keratic precipitates when present

COURSE OF UVEITIS

Forty patients (38.5%) had acute uveitis, 34 patients (32.7%) had chronic uveitis while 30

(28.8%) had recurrent uveitis.

Figure-34 Course of uveitis

Figure-35 Final Laterality

ANATOMICAL DIAGNOSIS OF UVEITIS

Of the 104 patients in the study, anterior uveitis was found in 59 patients (56.7%), posterior

uveitis in 21 patients (20.2%), panuveitis in 14 patients (13.5%) and intermediate uveitis in 8

patients (7.7%). Scleritis was found in 2 patients (1.9%). Eighty three patients (80%) had

anterior chamber inflammation irrespective of the anatomical diagnosis, of these, 20 patients

(19%) were pure iridocyclitis.

Figure-36 Anatomical diagnosis

Among the 44 patients with first episode of uveitis, 43% had anterior uveitis, 34% had posterior

uveitis, 18% had panuveitis and 4.5% had intermediate uveitis.

Figure-37 Anatomical diagnosis in patients with first episode

Course of uveitis in anatomic groups

Figure-38 Course of uveitis

Posterior uveitis

Of the 21 patients with posterior uveitis, 8 patients (38%) had multifocal choroiditis.

Figure-39 Types of Posterior uveitis

Vasculitis associated with uveitis

In the study, out of 104 patients, 9 (8.7%) patients were found to have vasculitis associated

with uveitis. Three patients had infectious uveitis - 2 patients had TB associated posterior

uveitis and 1 patient had CMV retinitis. Four patients had non-infectious uveitis, one patient

each with diagnosis of sarcoidosis, Behcet’s disease, VKH and Eales disease.

Figure-40 Types of Vasculitis associated with uveitis

SCLERITIS

In the study, 7 (6.7%) patients had scleritis. Three patients had anterior scleritis of idiopathic

origin. Two patients had posterior scleritis of idiopathic etiology and two had VKH related

posterior scleritis.

Figure -41 Types of scleritis associated with uveitis

Relevant Investigation to confirm diagnosis

Twelve patients (11.5%) had positive relevant investigations to confirm the diagnosis of

uveitis. Of these 7 patients had infectious etiology such as tuberculosis, leprosy, CMV and

toxoplasmosis. 5 patients whose investigations positive were of non-infectious etiology such

as HLA-B27, HLA-B51 and rheumatoid factor.

Figure-42 Investigation
ETIOLOGICAL DIAGNOSIS

Figure-43 Etiological diagnosis

Figure-44 Etiological diagnosis in anatomical subgroups

In our study, (figures 43&4 44) 59 patients had anterior uveitis. Of these, 23 (39%) patients

had idiopathic uveitis, 19 (32%) patients had infectious cause for uveitis and 17 (29%) patients

had non-infectious cause for uveitis.

Eight patients were diagnosed to have intermediate uveitis. Of these 7 had idiopathic uveitis

and 1 had non-infectious cause for uveitis.

Twenty one patients were diagnosed to have posterior uveitis. Of these, 13 (62%) patients had

infectious cause for uveitis, 6 (29%) due to a non-infectious cause and 2 (9.5%) due to

idiopathic etiology.

Fourteen patients were diagnosed to have panuveitis. Of these, 8 cases were due to a non-

infectious cause, 4 due to idiopathic and 2 cases due to infectious etiology.

DEFINITIVE ETIOLOGICAL DIAGNOSIS

Infectious uveitis

Of the 104 patients, 15 (14.4%) patients had viral cause for uveitis, 8 (7.7%) patients

tuberculous associated and 5 (4.8%) patients had toxoplasmosis associated uveitis.

Figure-45 Infectious uveitis

Non-infectious etiologies with known systemic associations

In the study, among the non-infectious etiological factors, 9 % cases were due to VKH, 6

(5.8%) HLA-B27 related, 5 (5%) JIA associated and 3(3%) sarcoidosis related uveitis. One

patient had sympathetic ophthalmia. Four patients had connective tissue disorder related uveitis

Figure-46 Non-infectious uveitis

Childhood uveitis

Figure-47 Childhood uveitis

There were 10 (9.6 %) children in the study ranging from 3 years to 16 years.
Of these, 4 children had Juvenile Idiopathic Arthritis (JIA) associated anterior uveitis

(2 unilateral and 2 bilateral). JIA patients did not have visual impairment in our study.

Three children had idiopathic uveitis

Three year old girl with unilateral acute anterior uveitis (affected eye visual acuity – 3/60) Six

year old girl with unilateral acute pan uveitis (affected eye visual acuity – HM)

Sixteen year old boy with bilateral chronic anterior uveitis and complicated cataract (visual

acuity RE-6/24, LE-PL positive)

HLA-B27 associated uveitis – One 15 year old boy with unilateral anterior uveitis. Patient did

not have visual impairment (visual acuity – 6/6).

Toxoplasma uveitis – One 16 years old girl with unilateral posterior uveitis with mild visual

impairment (visual acuity – 6/18).

Sarcoidosis associated uveitis – One 12 years old patient with bilateral posterior uveitis and

moderate visual impairment (visual acuity both eyes – 6/36).

HLA-B27 associated anterior uveitis

There were 6 patients diagnosed with HLA-B27 associated anterior uveitis. The course of

uveitis was recurrent in 3 patients, acute in 2 and chronic in 1 patient. Four patients had

unilateral involvement with no visual impairment and two patients had bilateral affection with

one of them having mild visual impairment

Figure-48 HLA-B27 associated anterior uveitis

.
Uveitis in patients with Tuberculosis

There were 8 patients with tuberculosis associated uveitis in our study. Four of them had

anterior uveitis and the other four had posterior uveitis. One of the patients with posterior

uveitis had severe visual impairment (visual acuity in the affected eye – 1/60).

Uveitis in patients with HIV infection

There were 5 (4.8%) patients with HIV infection in this study group. Two patients had

bilateral viral anterior uveitis and 3 had Cytomegalovirus (CMV) retinitis (two unilateral and

one bilateral posterior uveitis). Chart-49 Uveitis in patients with HIV infection
 Chart-50 Complications during the course of treatment

Of the 104 patients, 54 of them developed complications of uveitis or the medication.

Cataract was the most common complication 13 patients had developed cataract and

another eight underwent cataract surgery. Other major complications were cystoid macular

edema (14 patients) and secondary glaucoma (7 patients)

DISCUSSION

The uvea constitutes the vascular coat of the eye and inflammation of this structure is

broadly defined as uveitis. This disease can be broadly classified based on etiology, location

and clinical course of the disease. Uveitis can be an entity on its own involving only one eye

or both eyes or a manifestation of a systemic disease. (115)

The pathogenesis of uveitis is multifactorial and involves different inflammatory

pathways and defects in the regulatory mechanisms of the immune system. Corticosteroids is

the cornerstone drug in the management of uveitis. (116) but with the increasing knowledge of

the pathogenesis of the immune system, targeted biologic therapies are being developed. (117)

Uveitis is currently reported to be the third leading cause of blindness worldwide. It

currently accounts for 10% of preventable vision loss in the United States and 15% worldwide.

(23,118) Factors affecting the development of uveitis widely vary across the world due to

environmental changes like climate, topography and geography, socioeconomic differences in

the population, cultural differences and professional differences. A large series from south

India reported their experience over 6 years and compared it to reports from around the world.

In their hospital based data, uveitis was seen in 0.8% of the patients. The common confirmed

etiological diagnoses among infectious causes were leptospirosis, tuberculosis and herpes

related disease in adults and parasitic uveitis in children. (107)

The age of patients in our study ranged from 3 to 87 years. The majority of the patients

in the study ranged in the age group from 31-40 years (29%) followed by 41-50 years (20%)

and 51-60 years (16%) which is similar to multiple published studies. (5,6,41) Gupta A et al

in a retrospective study done north India from 1996-2001 reported ages ranging from 1.5 years

to 75 years with mean age of 34.4 years. Das D et al in study done North east India showed a
range of 2 to 61 years. Yang P in Chinese study in 2005 reported mean age at presentation as

33.8 +/- 16.5 years. (119)

Gender distribution was almost equal in our study, 51% females and 49% in males.

Pacific Ocular inflammation study (27) had shown that there was no difference in the incident

rates between males and females. With few exception, most of the surveys from developed

countries reported equal gender distribution or a slight predominance of women. Merrill PT et

al reported a female to male ratio of 1.6:1 referral population with a high percentage of African

Americans.(120) In a recent study by Luca C et al showed female to male ratio of 1.44:1 among

uveitis patients from northern Italy.(121) Male predominance is noted in studies from

developing countries including India. Female to male ratio 1:2 reported from Northeast India,

to almost 1:3 from south India.(6,28,41,107) Factors contributing to such differences that are

mentioned are complex and the most common being the fact that in developing countries males

may have more access to care and adequate medical treatment if they are ill (122). Our gender

distribution contrary to the trend in south India might be due to better access health care.

Family history of connective tissue disorders was positive in 3.8% patients. Of these,

one patients with HLA B-27 uveitis had family history of unspecified connective tissue and

uveitis while another patient had family history of Psoriasis. Most forms of non-infectious

uveitis occur within families and are polygenic with complex inheritance patterns. Many are

found to occur in families in which clustering of other autoimmune diseases have been

observed. Almost all polygenic diseases have a HLA genetic association. Some association can

be strong as in HLA-A29 with birdshot chorioretinopathy and HLA-B27 with Acute anterior

uveitis. However majority of people with positive markers do not develop Uveitis also (123–

127)
 Visual disturbance (87.5%) was the most common symptom of uveitis followed by

redness, pain, photophobia and watering respectively. Patients presented with blindness in

4.5% patients and 20% patients had moderate to severe visual impairment in the first episode.

Twenty five percent of the blindness in developing countries has been attributed to uveitis. (19)

Bilateral eye involvement was found in 75% of non-infectious disease and 55% of

idiopathic cases. Systematic review by Rathinam et al showed that in overall rates unilateral

and bilateral diseases are similar but non-infectious/auto immune entities tends to affect both

the eyes (107)

Anterior uveitis was the commonest uveitis (56.7%) in our study. Our study had a

higher percentage of anterior uveitis cases compared to other studies done in India (39-49%)

and other Asian studies (28-50%).(5,6,29) Acute unilateral non-granulomatous uveitis

occurred more frequently. Uveitis due to infectious etiologies were present in 33% of study

patients. Majority of the cases did not have any known systemic association or infection

(idiopathic, 37%). These findings are similar to the data from studies done by Rathinam et al

(28) from south India in the same state as our centre. However, in a prospective study conducted

in North Indian referral centre from 2011-2013 by Venkatesh, et al, they found that, out of 980

patients, 42% patients had anterior uveitis and majority due to idiopathic cause.(26)

There were total of 59 cases with anterior uveitis. Idiopathic anterior uveitis was seen

in 39% of the patients. This was the commonest followed by viral (20%), HLA-B27 (10%) and

TB (7%). Kalpana Babu, et al reported clinical profile of polymerase chain reaction proven

viral anterior uveitis. Out of 36 patients, 24 patients had Varicella Zoster, 7 had HSV,3 CMV

and 2 Chikungunya. Increase in IOP was seen in 28% of cases. Recurrence was seen in 61%

cases. (4)
 Idiopathic etiology accounted for majority of patients with anterior uveitis (39%) and

intermediate uveitis (88%), Only 28.6% of panuveitis and 9.5% cases of posterior uveitis were

idiopathic. while infectious caused 62% of posterior uveitis, 32% of anterior uveitis and 14%

of panuveitis. Non-infectious etiologies with known systemic association were the largest

etiological group in panuveitis (57%). Non-infectious etiologies caused 29% of anterior

uveitis,12.5% of intermediate uveitis and 29% of posterior uveitis

Isolated intermediate uveitis accounted for only 7.7% cases of all the patients in our

study. Seven patients had no identifiable cause and one was diagnosed to have Behcet’s

disease. From reports around the world, rates of intermediate uveitis, ranges from 1.4-22%.

Studies in India, found intermediate uveitis in the range of 9.5-17.4% among uveitis patients.

(6,26,28) From systematic reviews around the world, 60-100% intermediate uveitis was found

to be idiopathic. (29)

Twenty-one patients (20%) were diagnosed with posterior uveitis in our study. The

commonest identifiable causes for the inflammation were toxoplasmosis (24%), tuberculosis

(19%) and CMV (14%). In 10% of the cases no cause could be identified

Panuveitis was seen in 13.5% of the patients in our study. Among those diagnosed with

panuveitis, more than 70% had a specific etiology and 29% had no identifiable cause. Those

with specific diagnoses were as follows - VKH (50%), viral (14%) and sympathetic ophthalmia

(7%). Though worldwide, idiopathic uveitis is the most common cause of panuveitis, we found

that 50% of the patients with panuveitis had VKH in our study profile, as compared to 45%

reported by Das et al from hospital based study in North-east India. (128)

 Infectious pathology was detected in 32.7% of patients in our study. Common etiology

spectrum included viral uveitis (14%), tuberculosis (7.7%), toxoplasma (4.8%), CMV (2.9%)

and syphilis (1.9%).

Among patients with uveitis of non-infectious etiology, 43% had systemic association.

In our study, the spectrum of systemic diseases included Vogt-Koyanagi Harada disease, HLA-

B27 related, sarcoidosis, Juvenile idiopathic arthritis, Behcet’s disease, HLA-B51 related,

Rheumatoid arthritis, Spondyloarthropathy, Reiter’s and undifferentiated connective tissue

disorder related. A recent study reported by Gaurav Mathur, et al (1) reported that in non-

infectious anterior uveitis patients, 33% had HLA-B27 positive, 14% had spondyloarthropathy.

Based on the frequency of presentation, patients with non-infectious and idiopathic

uveitis had multiple episodes of uveitis. Recurrence rates was highest in those with idiopathic

(68%) etiology. They were also seen in non-infectious etiology (56%) and infectious etiology

(47%). The recurrence rates for were highest in HLA-B27 related disease (83%), followed by

VKH (56%), viral (47%) and tuberculosis (72%). In those with chronic uveitis, idiopathic

(32%) etiology was the commonest cause of recurrence, followed by TB (12%), viral (9%) and

VKH (9%).

High Intraocular pressure was found in 15 patients in our study. Seven patients out of

12 patients with anterior uveitis of viral etiology had high IOP. In a review by Uwe Pleyer et

al that Herpes simplex virus anterior uveitis caused raised intraocular pressure (IOP) in 38%–

90% of eyes. While with Herpes VZV IOP was elevated in 40–75% of cases. In acute CMV

anterior uveitis, mean presenting IOP was 50 mmHg, The IOP may be elevated in Chronic

CMV anterior uveitis but is not as high as that in the acute disease (129)
 Relevant investigations were positive in 16% patients which included HLA-B27

associated uveitis and JIA associated uveitis. Some diseases were diagnosed with specific

diagnostic criteria and therefore did not require extensive investigations.

Childhood uveitis accounted for 9.6 % of cases in our study. The commonest cause was

juvenile idiopathic arthritis related uveitis and was seen in 4 children. Three children had

idiopathic uveitis, one case each of HLA-B27 associated uveitis, sarcoidosis and toxoplasmosis

constituted the other three patients. In our study, anterior uveitis was the commonest (78%)

among children. Acute uveitis was seen in 7 patients while 3 children had chronic uveitis and

one with recurrent disease. Mild visual impairment was found in one patient and two of them

had moderate visual impairment. Anju Gupta, et al (18) reported that childhood uveitis

accounted for 5-10% of all uveitis and that anterior uveitis was the commonest. Common

etiologies of childhood uveitis were reported as JIA (11.6-30%), idiopathic, viral and

toxoplasmosis. JIA is the commonest cause of anterior uveitis in children. (18,130,131)

Systemic steroid was used for treatment in 27% cases of uveitis. Immunomodulators

along with topical and systemic steroids were used in 29% patients. Immunomodulators were

prescribed in 22.5% acute cases, 38% chronic cases and 43.4% recurrent cases of uveitis.

Corticosteroids are the mainstay of treatment in uveitis. Immunosuppressives have

revolutionized treatment of recurrent and chronic uveitis. More targeted treatment options

should be available in future as we get a better understanding of immunology and uveitic

diseases.

Of the 104 patients, 54 of them developed complications at presentation and or during

the course of uveitis treatment. Complicated cataract was seen in 38% cases. Eight patients

needed cataract surgery. Other complications included: cystoid macular edema (CME)-(25%),
secondary glaucoma (13%), retinal detachment (5%), steroid response (7%) and band shaped

keratopathy (4%).

Epidemiological and etiological patterns of uveitis in different regions of the world

have shown that there are similarities and differences in the pattern of uveitis influenced by

geographical, environmental and genetic patterns.

Most literature on hospital based uveitis profile studies which considered the full

spectrum of uveitic cases, occurred before standardization of uveitis nomenclature in 2005.

Many of them did not state the criteria that were used to diagnose uveitis. Hence, comparing

the results becomes difficult. Multiple etiologies are associated with uveitis. A meticulous

history taking, clinical examination, investigation would help in the diagnosis of the type and

severity of uveitis. Early initiation of treatment, compliance and regular follow-up is essential

to inhibit the progression of disease and relief of symptoms, thereby reducing the morbidity

associated with uveitis. The management of uveitis is usually a team work involving

ophthalmologists, rheumatologists and infectious disease specialists.

LIMITATIONS OF THE STUDY

1. While there is no ambiguity in the diagnosis of active uveitis, diagnosis of inactive and past

uveitis on the basis of history and clinical examination is not so easy especially when there

are no associated systemic signs and symptoms at presentation.

2. History suggestive of past uveitis elicited from a patient, may be very subjective, as ocular

pain, discomfort and redness may be present in many ocular conditions other than uveitis.

3. There is a high chance recall bias in family and past history.

4. The missing information, if not distributed randomly, we will miss significant risk factors.

5. The study is subject to confounding factors. Other risk factors may be present that were not

studied.

6. We can only determine the association and not the causation of all patients with uveitis in

view of the expensive tests and unavailability of all tests.

CONCLUSIONS

 The age group of 31 – 40 years had the highest prevalence of uveitis. Thus, uveitis may

have a strong socioeconomic and quality of life impact because it often affects younger

working-age patients

 Gender distribution was almost equal in our study.

 Visual disturbance was the most common symptom of uveitis

 Family history of connective tissue disorders was positive in 3.8% patients

 History of systemic tuberculosis was elicited in 2.9% patients.

 Systemic illness caused uveitis in 43% of patients with non-infectious etiology.

 Patients with non-infectious and idiopathic uveitis had a higher recurrence rate of

inflammatory episodes.

 In patients with chronic uveitis idiopathic cause was the commonest

 Bilateral eye involvement was found in 75% of non-infectious disease and 55% of

idiopathic cases.

 Idiopathic etiology accounted for majority of anterior and intermediate uveitis while

infectious etiology (62%) dominated the posterior uveitis group.

 Idiopathic anterior uveitis was the commonest of all presentations. Viral uveitis was the

commonest cause of infectious anterior uveitis

 Toxoplasmosis and tuberculosis were the commonest cause of posterior uveitis

 Vogt Koyanagi Harada disease was the commonest cause of panuveitis.

 Childhood uveitis accounted for 7.6%of all uveitis cases. Commonest cause was Juvenile

idiopathic arthritis related uveitis.

 Viral etiology was the commonest cause of those patients with a high intraocular pressure

during the episode of uveitis.

 HLA-B27 was the commonest positive investigation in the non-infectious uveitis group.

 Systemic steroid was used for treatment in 27% cases of uveitis.

 Immunomodulators along with topical and systemic steroids were used in 29% patients.

 Immunomodulators were prescribed in 22.5% acute cases, 38% chronic cases and 43.4%

recurrent cases of uveitis.

 Of the 104 patients, 54 of them developed complications of uveitis or the medication.

Complicated cataract was the most common complication.

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ANNEXURES
ANNEXURE-I IRB APPROVAL
ANNEXURE II

CLINICAL RESEARCH FORM: UVEITIS PROFILE STUDY

Serial no____ Schell hosp No: ___________________ CMC Hosp No:_________________

Name _______________ Age___year(s)

Occupation______________Residence_____________

Family history

Connective tissue disorder Y / N /NA _______________________________

Exposure to infection Y / N / NA ______________________________

Medical history

Systemic illness Y / N ____________________________

Exposure to infection Y/N ____________________________

Exposure to animals Y/N ____________________________

Ocular history

Number of episodes ______

Primary symptom at presentation

Laterality – Unilateral/ Right / Left/ Bilateral/ Alternating

(RE) Redness / Pain / Photophobia / Epiphora / Visual disturbances

(LE) Redness / Pain / Photophobia / Epiphora / Visual disturbances

Visual disturbances: Decreased vision / Diffuse blur/ Scotomata (central / Peripheral)

Floaters / Metamorphopsia / Micropsia / Macropsia.

Chief complaint, reason for visit

Laterality – Unilateral/ Right / Left/ Bilateral/ Alternating

(RE) Redness / Pain / Photophobia / Epiphora / Visual disturbances

(LE) Redness / Pain / Photophobia / Epiphora / Visual disturbances

Visual disturbances: Decreased vision / Diffuse blur/ Scotomata (central / Peripheral)

Floaters / Metamorphopsia / Micropsia / Macropsia.

 Associated symptoms_______________________________________

Ocualar injury (Y / N ) Right______________ Left ____________

Treatment history

Topical medication /

Topical + Systemic /

Topical +Systemic + Immunomodulators/

Antibiotics/ ATT/ Acyclovir

Compliance of the patient: GOOD/ POOR

Response to treatment:

Improvement / No improvement /

Worsened on treatment/ Worsened off treatment.

Treatment complications: Y / N Steroid response/ Cataract/ Others___________________

Systemic evaluation

Skin _______________________ CVS ________________________

RS ________________________ CNS_________________________

Joints ______________________ Fever ( Y / N )

Hearing loss ( Y / N )

Lymphadenopathy ( Y / N )

Ocular examination

Visual acuity: Right eye _________ Left eye_______

Scleritis Y / N (Anterior / Posterior )

Anterior uveitis ( Y / N ) (With spillover / Without spill over)

Granulomatous / Non granulomatous

Intraocular pressure: Low /Normal/ High / Steroid Response

Acute/ Chronic / Recurrent Unilateral/ Unilateral alternating / Bilateral

Anatomical diagnosis

Anterior uveitis Iritis / Iridocyclitis/ Anterior cyclitis

Intermediate uveitis Pars planitis /Posterior cyclitis / Hyalitis

Posterior uveitis Focal /multifocal / diffuse Choroiditis

Chorioretinitis /Retinochoroiditis /RetinitisNeuroretinitis

Panuveitis

Vasculitis: (Y / N ): Systemic non infectious / Infectious / Ocular disorder

Etiological diagnosis/ differential diagnosis: Infectious / Non Infectious / Idiopathic

Relevant investigations: (positive/negative)

Definitive diagnosis

Final Treatment :

Topical steroids /

Topical and systemic steroids /

Topical and systemic steroids + immunomodulators/

Antibiotics / ATT /
ANNEXURE-III DATA SPREAD SHEETS