The Great Mimicker

Minimal
History
anterior
of papulomacular
chamber reaction
rash with
with genital
papillitis,
ulcer
retinitis
with positive
and vasculitis
46 year old male with insidiousserology
progressive dimunition of central vision
THE GREAT MIMICKER
Ritweez Sahu
SYPHILIS
o OBLIGATE INTRACELLULAR GRAM NEGATIVE

SPIROCHAETE, TREPONEMA PALLIDUM.
o CROSSES INTACT MUCOUS MEMBRANES OR VIA
MICROABRASIONS IN THE SKIN.
o VERTICAL TRANSMISSION
o OTHER DIRECT MODES OF INOCULATION
o 20-70% CHANCE OF HIV COINFECTION
OCULAR SYPHILIS
Ocular structure Ocular incolvement Ocular structure Ocular incolvement
Eyelid Chancre Optic Nerve Perineuritis
Gumma Anterior Optic Neuritis
Ulcerative blepharitis Retrobulbar neuritis
Conjunctiva Chancre
Neuroretinitis
Conjunctivitis
Papilledema
Orbit Periostitis
Gumma in EOM, Lacrimal gland or
within orbit
Cornea Ulcers Motility disorders Cranial Nerve palsies
Deep Punctate Keratitis
Interstitial Keratitis
Retina and Vitreous Chorioretinitis
Sclera Episcleritis
Necrotizing retinitis
Scleritis
Retinal vasculitis
Gumma
Iris Roseolae Vitritis
Hypertensive anterior uveitis Vascular Occlusion
(Granulomatous/non granulomatous)
Pupil Argyll Robertson Pupil
Glaucoma Secondary Glaucoma
POSTERIOR MANIFESTATIONS
Chorioretinitis +/-vitritis
o The frequency of different subtypes of • Syphilitic punctate inner retinitis
uveitis varies greatly amongst different • Acute syphilitic posterior placoid chorioretinitis
publications • Necrotizing retinitis akin to CMV/ARN
• Syphilitic outer retinopathy akin to AZOOR
o Posterior and panuveitis most common
presentations Vasculitis
• Arteritis
o Optic disc involvement/papiliitis • Phlebitis
• May be occlusive
Serous retinal detachment without intraocular inflammation
Neuroretinitis
João M.Furtado,Milena Simões,Daniel Vasconcelos-Santos et al.,Ocular syphilis,Survey of Ophthalmology,

Zhang, Ting, Zhu, Ying, Xu, Gezhi, Clinical Features and Treatments of Syphilitic Uveitis: A Systematic Review and Meta-Analysis, Journal of Ophthalmology, 2017, 6594849, 15 pages, 2017.
ACUTE SYPHILITIC POSTERIOR PLACOID
CHORIORETINOPATHY (APPC)
o Characteristic lesion of secondary syphilis

o Originally described by Donald Gass
o One or more yellowish placoid
like retinochoroidal lesions that are typically
located in the macular region.
o This lesion was and may result from an infection
with T. pallidum of the RPE layer in the posterior
pole or as a consequence of indirect immune-
mediated hypersensitivity
o Retinal vasculitis is often associated with a
"ground glass" retinitis, which is characteristic of
syphilis. Typically, arteries are more involved.
ACUTE SYPHILITIC POSTERIOR PLACOID
CHORIORETINOPATHY (APPC)
o Characteristic lesion of secondary syphilis

o Originally described by Donald Gass
o One or more yellowish placoid
like retinochoroidal lesions that are typically
located in the macular region.
o This lesion was and may result from an infection
with T. pallidum of the RPE layer in the posterior
pole or as a consequence of indirect immune-
mediated hypersensitivity
o Retinal vasculitis is often associated with a
"ground glass" retinitis, which is characteristic of
syphilis. Typically, arteries are more involved.
PUNCTATE INNER RETINITIS
SYPHILITIC OUTER RETINOPATHY
PSEUDO-RETINITIS PIGMENTOSA
FAF
o Localized hyperautofluorescence in the

area of the lesion
o Focal intense hyperautofluorescence
corresponding to areas of RPE
irregularity
Browning DJ. Posterior segment manifestations of active ocular syphilis, their response to a neurosyphilis regimen of penicillin therapy, and the influence of human immunodeficiency virus status on response.
Ophthalmology. 2000 Nov;107(11):2015-23. doi: 10.1016/s0161-6420(00)00457-7. PMID: 11054325.
OCT
o Disruption of the inner segment/outer

segment junction and ELM
o Nodular thickening of the RPE and
excresences into outer retina
o subretinal fluid
o punctate hyperreflectivity of the choroid
Pichi, F., Ciardella, A. P., Cunningham, E. T., Morara, M., Veronese, C., Jumper, J. M., … Nucci, P. (2014). SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS IN PATIENTS WITH ACUTE SYPHILITIC
POSTERIOR PLACOID CHORIORETINOPATHY. Retina, 34(2), 373–384.
FFA
o Fluorescein angiography showed early

central hypofluorescence followed by
progressive hyperfluorescence in the
area of the lesion
o associated with an active leading edge
identified angiographically as an
increased late leakage and staining at
the margin
o Variable or punctate hypofluorescence
producing a classic “leapord spot”
Pichi, F., Ciardella, A. P., Cunningham, E. T., Morara, M., Veronese, C., Jumper, J. M., … Nucci, P. (2014). SPECTRAL DOMAIN OPTICAL COHERENCE TOMOGRAPHY FINDINGS IN PATIENTS WITH ACUTE SYPHILITIC POSTERIOR
PLACOID CHORIORETINOPATHY. Retina, 34(2), 373–384.
SUN WORKING GROUP
Classification Criteria
Uveitis with a compatible uveitic presentation, including
• Anterior uveitis OR
• Intermediate uveitis or anterior/intermediate uveitis OR
• Posterior or panuveitis with one of the following presentations
• Placoid inflammation of the retinal pigment epithelium or
• Multifocal inflammation of the retina/retinal pigment epithelium or
• Necrotising retinitis or
• Retinal vasculitis
Evidence of infection with Treponema pallidum, either
• Positive treponemal test and non-treponemal test

• Positive treponemal test with two different treponemal tests
Excluding history of treatment for syphilitic uveitis

SEROLOGY
ANTIBODIES
o Treponemal antibodies (FTA ABS) o Non treponemal (RPR)(VDRL)

(TPHA)(TPPA)(MHA-TP) (TP-EIA)
o Titres revert on treatment
o They remain positive for life following
infection and remained positive after o False negative- early/primary infections
successful treatment.
o False positive- TB, Ricketssia, Leprosy
o Positive result does not inform the
recency or activity of infection
o False-positive indicates SLE or other
other spirocheteal infections such as
yaws, bejel and pinta.
TREATMENT
OCULAR SYPHILIS/NEUROSYPHILIS
o THE CDC RECOMMENDED o CEFTRIAXONE 1–2 G

TREATMENT NEUROSYPHILIS – INTRAMUSCULARLY (IM/IV) DAILY
FOR 14 DAYS
o AQUEOUS CRYSTALLINE PENICILLIN G
(BENZYLPENICILLIN), 18–24 MILLION o COURSE OF ORAL CORTICOSTEROIDS
UNITS DAILY, GIVEN AS 3–4 MILLION SUCH AS PREDNISOLONE BEGINNING
UNITS IV EVERY 4 H OR AT 1 MG PER KG AND TAPERING
CONTINUOUSLY INFUSED, FOR 10–15 OVER A MINIMUM OF 6-8 WEEKS
DAYS JARISCH-HERXHEIMER
HYPERSENSITIVITY RESPONSE
WHO NEEDS LUMBAR
PUNCTURE.
o 60-70% OF PATIENTS WITH OCULAR SYPHILIS HAVE

CSF ABNORMALITIES
o ALL PATIENTS OF OCULAR SYPHILIS ARE TREATED AS
IF THEY HAVE NEUROSYPHILIS
o NEUROLOGICAL AND OTOLOGICAL EVALUATION IS
NECESSARY AS THEY MIGHT REQUIRE ALTERNATE
MANAGEMENT.
o LUMBAR PUNCTURE NOT NECESSARY IN ALL
Lapere S, Mustak H, Steffen J. Clinical manifestations and cerebrospinal fluid status in ocular syphilis. Ocul Immunol Inflamm.
2019;27:126–30.
The Ophthalmologist has a major role in the diagnosis and
management of syphilis.
In patients with uveitis and optic neuritis, there is very rarely a good
reason not to test for syphilis.
Multidisciplinary management in concurrence with Infectious/Veneral

diseases physicians, Neurologist, Otologist
THE PHYSICIAN WHO KNOWS
SYPHILIS, KNOWS MEDICINE
SIR WILLIAM OSLER

The Great Mimicker

Uploaded by

Copyright:

Available Formats

The Great Mimicker

Uploaded by

Document Information

Original Title

Copyright

Available Formats

Share this document

Share or Embed Document

Sharing Options

Did you find this document useful?

Is this content inappropriate?

Copyright:

Available Formats

The Great Mimicker

Uploaded by

Copyright:

Available Formats

Minimal

o OBLIGATE INTRACELLULAR GRAM NEGATIVE

João M.Furtado,Milena Simões,Daniel Vasconcelos-Santos et al.,Ocular syphilis,Survey of Ophthalmology,

o Characteristic lesion of secondary syphilis

o Characteristic lesion of secondary syphilis

o Localized hyperautofluorescence in the

o Disruption of the inner segment/outer

o Fluorescein angiography showed early

Evidence of infection with Treponema pallidum, either

• Positive treponemal test and non-treponemal test

Excluding history of treatment for syphilitic uveitis

o Treponemal antibodies (FTA ABS) o Non treponemal (RPR)(VDRL)

o THE CDC RECOMMENDED o CEFTRIAXONE 1–2 G

o 60-70% OF PATIENTS WITH OCULAR SYPHILIS HAVE

Multidisciplinary management in concurrence with Infectious/Veneral

SIR WILLIAM OSLER

You might also like