Introduction

OCULAR MANIFESTATIONS

SYSTEMIC DISEASES
Introduction

CATEGORIES OF SYSTEMIC
DISEASE
•  Congenital
•  Traumatic
•  Vascular
•  Neoplastic
•  Autoimmune
•  Idiopathic
•  Infectious
•  Metabolic/Endocrine
•  Drugs/Toxins
Introduction

EYE EXAMINATION

•  Vision
•  External
•  Pupils
–  Relative afferent pupillary defect
•  Motility
•  Anterior segment exam
•  Dilated ophthalmoscopy
•  Visual fields
Congenital Disorders

CONGENITAL DISORDERS

•  Down syndrome
•  Marfan syndrome
•  Myotonic dystrophy
•  Tuberous sclerosis
•  Neurofibromatosis
•  Congenital metabolic disorders
–  Lysosomal storage
–  Carbohydrate metabolism
Congenital Disorders

NEUROFIBROMATOSIS NF1

Café-au-lait spots Iris Lisch nodules

Traumatic Disorders

Shaken baby syndrome: multiple retinal hemorrhages

Vascular Disorders

SYSTEMIC HYPERTENSION:
FACTORS AFFECTING RETINAL
•  Severity
ARTERIOLES
•  Duration
Vascular Disorders

Malignant hypertension
Vascular Disorders

Embolic Disease

Sites of emboli to the Cholesterol embolus

ophthalmic circulation (Hollenhurst plaque)
Vascular Disorders

Left eye with superotemporal branch retinal artery occlusion

Vascular Disorders

CRAO

“Cherry red spot” in CRAO Normal fundus

Vascular Disorders

AMAUROSIS FUGAX
"Fleeting Blindness"
•  Monocular dimming of vision
•  Temporary arterial obstruction
•  Sudden, transient, painless visual loss
Vascular Disorders

AMAUROSIS FUGAX:
A MULTIDISCIPLINARY
• 
EVALUATION
Cardiovascular
•  Cerebrovascular
•  Ophthalmologic
Vascular Disorders

Talc embol in IV drug abuser

Vascular Disorders

Central retinal vein occlusion (CRVO)

Vascular Disorders

MIGRAINE:
VISUAL SYMPTOMS
Transient homonymous hemianopia
•  Scintillations
•  Transient
obscuration of
vision
Left eye Right eye
•  Transient
homonymous
hemifield loss
Scintillations
Vascular Disorders

MIGRAINE: TYPES

•  With headache
–  Classic
–  Common
–  Complicated

•  Without headache
–  Acephalgic
Vascular Disorders

MIGRAINE: TREATMENT

•  Prevent frequent attacks

•  Examine for visual loss
•  Consider discontinuing oral contraceptives
Vascular Disorders

BLOOD DYSCRASIAS
WITH OCULAR
•  MANIFESTATIONS
Hyperviscosity syndromes
•  Thrombocytopenia
•  Anemia, including sickle cell anemia
Vascular Disorders

HYPERVISCOSITY
SYNDROMES:
•  OCULAR
Amaurosis fugax SYMPTOMS
•  Permanent visual loss
Vascular Disorders

Hyperviscosity dilated retinal veins,

retinal hemorrhages, and disc edema
Vascular Disorders

Leukemia
Vascular Disorders

Leukemic infiltration of the optic nerve

Vascular Disorders

SICKLE CELL RETINOPATHY

•  HbSC disease (most common form)

•  HbSS disease
•  Sickle thalassemia
Vascular Disorders

SICKLE CELL ANEMIA

Retinal arterial occlusions “Sea fan” neovascularization

Neoplastic Disorders

MALIGNANT NEOPLASMS
INVOLVING THE EYE
•  Primary ocular melanoma
•  Large cell lymphoma
•  Metastatic carcinoma
Neoplastic Disorders

METASTATIC CARCINOMA

•  Most common intraocular malignancy in

adults
•  May be asymptomatic
•  May produce decreased or distorted vision
Neoplastic Disorders

Iris metastasis from lung carcinoma

Neoplastic Disorders

Choroidal metastasis from breast cancer

Neoplastic Disorders

Ophthalmologists should
monitor ocular metastases
at regular intervals.
Autoimmune Disorders

AUTOIMMUNE DISORDERS

•  Connective tissue diseases

•  Thyroid eye disease
•  Myasthenia gravis
Autoimmune Disorders

CONNECTIVE TISSUE
DISORDERS
•  Dry eyes are the most common
manifestation (keratoconjunctivitis sicca)
•  Symptoms
–  Burning
–  Foreign-body sensation
–  Photophobia
Autoimmune Disorders

SJÖGREN’S SYNDROME

•  Dry eyes
•  Dry mouth
•  ± Connective tissue disorder
•  Specific antibodies
Autoimmune Disorders

DRY EYES: TREATMENT

•  Artificial tears
•  Lubricating ointment at night
•  Punctual occlusion
•  Environmental modification
•  Topical anti-inflammatory agents
Autoimmune Disorders

Severe tear deficiency: corneal ulceration and perforation

Autoimmune Disorders

ANKYLOSING SPONDYLITIS:
OCULAR SYMPTOMS
•  Photophobia
•  Redness
•  Decreased vision

If iritis, refer for evaluation

and treatment.
Autoimmune Disorders

Topical corticosteroids may

lead to serious ocular
complications.
Autoimmune Disorders

RHEUMATOID ARTHRITIS:
OCULAR MANIFESTATIONS
•  Dry eyes
•  Episcleritis
•  Scleritis
•  Corneal ulcers
•  Uveitis
Autoimmune Disorders

Episcleritis
Autoimmune Disorders

Scleritis
Autoimmune Disorders

Scleritis Necrotizing
scleritis
Autoimmune Disorders

Scleromalacia perforans
Autoimmune Disorders

Peripheral corneal ulceration

Autoimmune Disorders

Juvenile rheumatoid arthritis: band keratopathy

Autoimmune Disorders

IRITIS IN JUVENILE
RHEUMATOID ARTHRITIS
•  Few symptoms or signs
•  Usually chronic
•  Secondary cataract and glaucoma

Refer for periodic ophthalmic screening.

Autoimmune Disorders

SYSTEMIC LUPUS
ERYTHEMATOSUS: OCULAR
•  Dry eyes
MANIFESTATIONS
•  Scleritis
•  Peripheral corneal ulcers
•  Retinopathy and optic neuropathy
Autoimmune Disorders

Systemic lupus erythematosis:

Cotton wool spots and hypertensive retinopathy
Autoimmune Disorders

Systemic lupus erythematosis: optic nerve involvement

Autoimmune Disorders

POLYARTERITIS NODOSA:
OCULAR MANIFESTATIONS
•  Dry eyes
•  Corneal ulcers
•  Scleritis
•  Hypertensive retinopathy
•  Retinal vasculitis
Autoimmune Disorders

SARCOIDOSIS

•  More commonly affects African-Americans

and Hispanics
•  Ocular involvement in about 25% of
patients
Autoimmune Disorders

SARCOIDOSIS

Keratic precipitates in “Candlewax drippings” in

granulomatous uveitis choroiditis
Autoimmune Disorders

Refer patients with sarcoidosis

if ocular involvement is
suspected.
Treatment may include
corticosteroids.
Autoimmune Disorders

GIANT CELL ARTERITIS:

SYMPTOMS
•  Jaw claudication
•  Headache
•  Scalp tenderness
•  Fever
•  Weight loss
•  Fatigue
•  Myalgias
•  Acute visual loss
Autoimmune Disorders

Giant cell arteritis: ischemic optic neuropathy

Autoimmune Disorders

Giant cell arteritis: third cranial nerve involvement

Autoimmune Disorders

GIANT CELL ARTERITIS:

DIAGNOSIS
•  Clinical history
•  STAT ESR and CRP
•  Temporal artery biopsy
•  Fluorescein angiogram
Autoimmune Disorders

GIANT CELL ARTERITIS:

MANAGEMENT
•  Begin treatment immediately with high-
dose corticosteroids daily
•  Do not wait for results of temporal artery
biopsy
•  Ophthalmologist, PCP, and rheumatologist
manage jointly
Autoimmune Disorders

THYROID OPHTHALMOPATHY

•  Not always correlated with serum thyroid

levels
•  Can progress after thyroid function is
normal
Autoimmune Disorders

SPECTRUM OF THYROID
OPHTHALMOPATHY
•  Eyelid retraction
•  Exophthalmos (proptosis)
•  Extraocular muscle dysfunction
•  Corneal exposure
•  Conjunctival erythema
•  Optic nerve dysfunction
Autoimmune Disorders

THYROID OPHTHALMOPATHY:
OCULAR INVOLVEMENT

Widened palpebral fissure Soft tissue involvement

Autoimmune Disorders

THYROID OPHTHALMOPATHY:
ADVANCED

Inferior rectus muscle

Proptosis restriction
Autoimmune Disorders

THYROID OPHTHALMOPATHY:
SEVERE

Corneal damage Swollen optic disc

Autoimmune Disorders

THYROID OPHTHALMOPATHY:
COMPUTED TOMOGRAPHY OF THE
ORBIT

Proptosis Enlarged extraocular muscles

Autoimmune Disorders

THYROID OPHTHALMOPATHY:
TREATMENT OF CONGESTIVE
•  Tear substitutes
PHASE
•  Corticosteroids
•  Orbital irradiation or
surgical decompression
Autoimmune Disorders

THYROID OPHTHALMOPATHY:
TREATMENT OF CICATRICIAL
•  Lid surgery
PHASE
•  Muscle surgery
•  Orbital surgery
Autoimmune Disorders

MYASTHENIA GRAVIS:
SYSTEMIC INVOLVEMENT

Refer suspects for neurologic evaluation.

Idiopathic Disorders

Papilledema
Idiopathic Disorders

INTRACRANIAL
HYPERTENSION: CAUSES
•  Brain tumor
•  Meningitis
•  Venous sinus
thrombosis
•  Hydrocephalus
•  Idiopathic
intracranial
hypertension
(pseudotumor
cerebri)
Idiopathic Disorders

OPTIC NEURITIS

•  May be initial manifestation of MS

•  Acute, painful vision loss in one or both
eyes
•  Treat initial episode with intravenous
methylprednisolone
•  Interferon beta-1a may be of benefit
Infectious Disorders

AIDS: OCULAR
MANIFESTATIONS
•  Dry eye
•  Retinal cotton-wool spots
•  CMV retinitis
•  Kaposi’s sarcoma
(eyelid or conjunctiva)
Infectious Disorders

AIDS: Cotton wool patches

Infectious Disorders

AIDS: cytomegalovirus (CMV) retinitis

Infectious Disorders

CMV RETINITIS: THERAPY

•  HAART (Highly Active Anti-Retroviral)

Therapy
•  IV ganciclovir
•  IV foscarnet
•  Intravitreal ganciclovir
•  Implantable pellets of sustained-release
ganciclovir into the vitreous
Infectious Disorders

CD4 COUNTS

•  < 100 cells/ml:

Opportunistic infections (eg, CMV retinitis)
•  > 100 cells/ml:
Other ocular infections (eg, syphilis)
Metabolic/Endocrine Disorders

Diabetic retinopathy
Drugs/Toxins

SYSTEMIC MEDICATIONS
WITH SIGNIFICANT OCULAR
EFFECTS
•  Toxic Retinopathy
–  Thioridazine
–  Chloroquine
–  Hydroxychloroquine
–  Tamoxifen

•  Toxic Optic Neuropathy

–  Ethambutol
–  Isoniazid
–  Fluoroquinolones
Conclusion

OCULAR MANIFESTATIONS

SYSTEMIC DISEASES