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