Euretina Meeting 2013 Hamburg

Miles StanfordMedical Eye Unit

St Thomas’ HospitalLondon

Serpiginous choroiditis

Serpiginous choroiditis

• Rare

• Bilateral

• 40-60 years

• Mainly caucasian

• Slight preponderance for men

Serpiginous choroiditis - pathology

• Little available

• Widespread atrophy of photoreceptors, RPE and choriocapillaris

• Lymphocytic infiltration of the choroid

• Secondary choroidal neovascularisation

Serpiginous choroiditis – clinical features

• Unilateral decrease in central vision, metamorphopsia or scotoma

• Little anterior segment reaction• Lesions classically peripapillary and then

spread outwards• Disease progression is stepwise and

asymmetric• Eventually permanent scar and subretinal

fibrosis

Serpiginous – progression over 6 months

Serpiginous choroiditis – stepwise progression over 18 months

Fluorescein angiography showing early masking and late staining on the edge of a old

scar

Serpiginous – FFA staining at the edge of an old scar. These changes may be more evident on ICG

Serpiginous choroiditisdifferential diagnosis

• APMPPE• Myopia• Choroidal ischaemia• Sarcoidosis• Toxoplasma• Tuberculosis/syphilis• Metastases/lymphoma• Retinochoroidal dystrophies

Ampiginous choroiditis (mantoux 20mm, subsequently developed Eales’ disease)

Serpiginous-like choroiditis and TB• Presumed uveitis due to TB: All patients with 1 year

follow up, exclusion of other infections, +ve Mantoux, no recurrence after full anti TB treatment

• 26/192 (15%) patients with presumed TB-related posterior uveitis had serpiginous like choroiditis (OR 26; 95% CI 7.4-91.4)

• Sensitivity 14%: specificity 98%: positive predictive value 56%

• Not a good sign for screening but makes diagnosis 90% certain if positive

Gupta A et al Am J Ophthalmol 2010 149:562

Serpiginous-like choroiditis and TB

• 11/21 (52%) patients tested +ve with Quantiferon compared to 9% HC and 13% uveitis controls

• 3/11 improved with specific anti-TB treatment

• ?directly due to bacteria or allergic response

Mackensen F et al Am J Ophthalmol 2008 146;761

Serpiginous-like choroiditis and TB

• Comparison of 5 patients with serpiginous like (SLC) and classical serpiginous (SC)

• Patients with SLC were: - most likely to have come from a country

where TB endemic - To have unilateral multifocal disease with

significant vitritis - to have a positive PPD - to respond to tuberculostatic therapy

Arch Ophthalmol 2010 128: 853

Serpiginous choroiditisInvestigations

• FFA

• ICG

• OCT

• Electrodiagnostics

• Visual fields

• Mantoux/IFN gamma

Serpiginous choroiditis - complications

• CNVM occurs in 15-35%

• Usually arises from the edge of a scar but may be peripapillary

• Serous retinal or RPE detachments

• Subretinal fibrosis

• Rarely, CMO or NVs

Serpiginous choroiditisTreatment

• Goals of therapy are to control active lesions rapidly and to prevent further recurrences and progression

• Steroids – oral or pulsed

• Other immunosuppressives

• Infliximab

• Treatment for secondary neovascularisation

Serpiginous choroiditis - prognosis

• Very few long term studies

• Chronic, progressive disease in a stepwise manner

• Active lesions usually resolve over 3-6 months but may take longer

• Extrafoveal lesions may not give rise to symptoms and so pass unrecognised

Serpiginous choroiditis - Conclusions

• Rare, progressive disease of the middle-aged

• Must exclude TB especially if patient from endemic area

• Treat with standard immunosuppressives to control active lesions and prevent progression

• Potential for secondary CNVM