Ocular Syphilis
Nicholas P Jones
The Royal Eye Hospital
Manchester, UK
Syphilis and uveitis
• Congenital N
• Primary N
• Secondary (early, active) Y
• (late, latent) N
• Late (symptomatic) (Y)
Other secondary symptoms
• Headache
– Global, persistent
– Occasional meningism
• Malaise, tiredness
• Lymphadenopathy
• Condylomata lata
Ocular syphilis - manifestations
• Anterior uveitis/vitritis/panuveitis
Ocular syphilis - manifestations
• Retinitis/vasculitis
Ocular syphilis - manifestations
• Retinitis/vasculitis – with multifocal peri-
retinal “satellite” lesions
Ocular syphilis - manifestations
• Vitritis
• Papillitis
optic neuropathy
Ocular syphilis - manifestations
• Placoid
chorioretinitis
• HIV+ ?
Ocular syphilis – uncommon or
rare manifestations
• Acute interstitial keratitis/sclerokeratitis
• Neuroretinitis
• Retinal vein occlusion
• Exudative/serous retinal detachment
• Necrotising retinitis
When to suspect syphilis
• Any uveitis with:
– skin rash, especially involving palms/soles
or with mucosal ulcer
– headache
– history of sexually transmitted disease(s)
including known HIV
• Any retinitis or retinal vasculitis
• Any unresponsive uveitis
Posterior uveitis with skin lesions:
Differential diagnosis
• Behçet’s disease
• Sarcoidosis
Posterior uveitis with skin lesions:
Differential diagnosis
• Syphilis
• Lupus
Posterior uveitis with skin lesions:
Differential diagnosis
• Varicella
• Others
Uveitis with headache
• Behcet’s disease (idiopathic, encephalitis, aseptic meningitis)
• Syphilis (meningoencephalitis)
• APMPPE (meningism, cerebral vasculitis)
• Multiple sclerosis (focal demyelination)
• VKH (meningism [meningeal pigmented cells, pineal])
• Encephalitis (infective) – HSV, Lyme, Whipple’s, brucella
– in the immunodeficient: cryptococcus, toxoplasma
• Systemic vasculitis (cerebral vasculitis)
Syphilis - investigation
• Treponemal tests: – ELISAs including ICE, DBE
– FTA-ABS – less common
– TPHA, TPPA - less good
• Non-treponemal tests:
– Rapid Plasmin Reagent (RPR)
– Venereal Disease Research Laboratory (VDRL)
• quantitative (titre >1:4 shows current activity)
• confirms active infection
• monitors treatment progress
Syphilis - investigation
• Treponema pallidum cannot be cultured
• T pallidum pertenue (yaws) and other
endemic syphilis organisms are
immunologically identical
• Infection with T pallidum confers lifelong
positive treponemal test, but NOT
immunity: syphilis can be caught repeatedly
Interpreting syphilis tests
T pallidum PCR on intraocular fluid
• Sensitivity and specificity not ratified for
intraocular use
• TaqMan probe-enhanced real-time PCR
enhances specificity
• Vitreous may be significantly more
productive than aqueous
Refer to genitourinary
medicine clinic because:
• Supervised treatment
• Interpretation of repeated serology
• Investigation/counselling for other sexually
transmitted diseases including HIV/HepC
– HIV accelerates neurosyphilis
• Contact tracing and treatment
Exclude active neurosyphilis?
• Headache nonspecific
– (doesn’t indicate CNS infection)
• Exclude focal neurological signs
• CSF analysis if necessary: – WCC >20/microl, protein >45mg/dl
– VDRL +ve (not RPR - v. insensitive)
– Treponemal test +ve
– FTA-ABS +ve, TPHA+ve = 87% sensitive, 94% specific
• CT brain if necessary
Syphilis - treatment
• Regime as for presumed neurosyphilis:
– Procaine penicillin G 2.4MU/day I/M 17/7
– Probenecid 500mg QID oral 17/7
– Oral steroids to:
• treat sight-threatening uveitis (40-60mg/day)
• ameliorate Jarisch-Herxheimer reaction (20mg/day)
• Or: benzylpenicillin 18-24MU/day I/V 17/7
• Or: doxycycline 200mg BD 4/52
• Or: amoxycillin 2g TDS + probenecid 500QID 4/52
UK National Guideline 2002 for management of Late Syphilis (Assoc GUM)
Response to treatment
• May take weeks to settle, but often good VA
• Retinal atrophy more
extensive than areas of
active retinitis:
• Large visual field defects
• Nyctalopia
Post-treatment follow-up
• Monitor inflammation, symptoms, field
• Monitor RPR; 4-fold rise = re-infection – treatment response much slower, uveitis risk low
In conclusion:
• Syphilis is not uncommon: think of it!
• Take a sexual/STD history
• Serology is diagnostic - always include it if
syphilis is possible
• Always liaise with GUM physician
• Treatment curative, but visual recovery may
be delayed