Typically a bilateral, chronic, recurrent inflammation of the retinal pigment epithelium (RPE), choriocapillaris, and choroid
• Rare. The incidence and prevalence are not well-established.
• Most reports suggest that cases of serpiginous choroiditis constitute less than 5% of cases of posterior uveitis. Prevalence may be higher in India as one Indian study reported that serpiginous choroiditis made up 19% of all cases of posterior uveitis (1)[A].
• Tends to occur in healthy young to middle-aged adults, more commonly males
• No racial or familial predisposition exists.
No known risk factors
One Finnish study suggests that HLA-B7 is more prevalent among patients with serpiginous choroiditis.
Chronic, recurrent inflammation of the RPE, choriocapillaris, and choroid
• Typically presents with unilateral vision loss, distortion, or scotoma. In the majority of cases, both eyes will eventually be affected.
• The disease course is chronic and progressive with recurrences in both eyes.
• Anterior segment exam usually appears quiet with no inflammation.
• Dilated exam classically shows subretinal lesions, which originate in the peripapillary region and spread centrifugally in a serpentine pattern. Macular and amphigenous variants of lesions also exist (see below).
• The visual outcome is related to the proximity of the lesions to the fovea and to the development of choroidal neovascularization.
There are three main variants:
• Classic (peripapillary geographic)
– Accounts for 80% of cases
– Lesions begin as creamy subretinal infiltrates in the peripapillary region and spread centrifugally in a serpentine pattern.
– Active lesions will resolve in 6–8 weeks (with or without treatment), resulting in atrophy of the affected choroid and RPE.
– Recurrences generally occur at the edges of old scars and continue to spread in a serpentine pattern. Recurrences occur at variable intervals ranging from months to years.
– Studies have suggested that the final visual acuity is less than 20/200 in up to 25% of treated eyes.
– Clinical and angiographic features are similar to classic serpiginous except that the lesions begin in the macula and are not continuous with the optic nerve.
– Visual prognosis is worse than classic serpiginous because of earlier foveal involvement and higher risk of choroidal neovascularization.
• Atypical variants
– Amphigenous–-There is a subset of patients initially diagnosed with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) who eventually developed serpiginous choroiditis. These patients tend to have less foveal involvement.
– Relentless placoid chorioretinitis—There is a subset of patients with clinical features similar to amphigenous; however, the lesions affect the entire retina from the posterior pole to the periphery.
DIAGNOSTIC TESTS & INTERPRETATION
• Fluorescein angiography (FA) and indocyanine green angiography(ICGA)
– Active lesions (generally at the edges of older lesions) show early hypofluorescence and late staining on FA.
– Old, atrophic lesions show early hypofluorescence and progressive hyperfluorescence at the margins with late staining of the underlying sclera on FA.
– ICGA, which focuses on choroidal circulation, shows hypofluorescence of the lesions.
– The reason for hypofluorescence of the lesions on FA and ICGA is not entirely known. Possible reasons include choroidal nonperfusion or blockage of perfusion by inflammatory cells.
• Visual fields
– Visual field testing demonstrates scotomata corresponding to the lesions, which may become less dense over time.
• Multifocal choroiditis
• Choroidal ischemia
Many treatment regimens have been used but given the rarity of the disease and the chronic nature, there is limited data on long-term efficacy.
– Systemic, peribulbar, and intravitreal steroids are effective in treating active lesions and reducing the period of active disease (2)[A].
– Relapses are common during steroid tapers or after steroids are stopped.
• Immunomodulatory agents
– Various agents including methotrexate, cyclosporine A, azathioprine, and mycophenolate mofetil have been used for long-term management in steroid-dependent or resistant cases with good results. Some patients experience recurrences with tapering of immunosuppressive medications (3)[A].
– Alkylating agents (cyclophosphamide and chlorambucil) were shown to be effective in managing serpiginous choroiditis. However, their use in the current management of this condition is limited by potentially severe adverse effects.
• Frequent follow-up is necessary to diagnose and treat recurrences or choroidal neovascularization.
• Patients should be instructed to call for worsening of vision, metamorphopsia, or new scotomas.
• The disease process is chronic and progressive with multiple recurrences.
• Visual prognosis is guarded due to possible scarring/atrophy of the fovea and secondary choroidal neovascularization.
• The most common complication of serpiginous choroiditis is choroidal neovascularization, which occurs in 13–35% of patients.
• Reported complications include branch retinal vein occlusion, periphlebitis, pigment epithelial detachment, serous retinal detachment, cystoid macular edema, optic disc neovascularization, subretinal fibrosis, and anterior uveitis.
1. Lim WK, Buggage RR, Nussenblatt RB. Serpiginous choroiditis. Surv Ophthalmol 2005;50:231–244.
2. Akpek EK, Ilhan-Sarac O. New treatments for serpiginous choroiditis. Curr Opin Ophthalmol 2003;14(3):128–131.
3. Akpek EK, Baltatzis S, Yang J, et al. Long-term immunosuppressive treatment of serpiginous choroiditis. Ocul Immunol Inflamm 2001;9:153–167.