Serpiginous Choroidopathy DISEASE entity
Serpiginous choroidopathy (SC) is a rare, bilateral, chronic, progressive, recurrent inflammatory disease of the retinal pigment epithelium (RPE), choriocapillaris, and choroid of unknown etiology.
SC is a rare clinical entity causing less than 5% of posterior uveitis cases. It has a higher prevalence in men and affects young to middle-aged adults. No systemic disease associations have been identified.
Patients present with painless unilateral vision loss, metamorphosis, or central scotoma.
Although of unknown etiology, its origin is probably immunogenic since it seems to respond to treatment with corticosteroids and other immunosuppressants. Moreover, affected patients also show an increased frequency of HLA-B7 and retinal S-antigen associations.
Other pathogenic mechanisms have been proposed and there may be an association with SC with infectious etiologies such as Mycobacterium tuberculosis and herpes viruses but this remains to be proven. Alternatively, SC and tuberculosis-related SC may be different disease entities with overlapping features.
Serpiginous Choroidopathy Diagnosis
Serpiginous choroidopathy (SC) presents with gray-yellowish subretinal infiltrates that usually spread centrifugally from the peripapillary region in a serpiginous (snake-like) manner. Active lesions show a leading edge and resolve with subsequent RPE and choriocapillary atrophy.
Consecutive recurrences cause further atrophy leaving hypo- and hyper-pigmented lesions that spread irregularly over the posterior fundus. Although bilateral, the disease is often asymmetric with multiple lesions in different stages of resolution in both eyes.
Recurrences have variable intervals that range from months to years. Anterior chamber and vitritis are minimal.
The former begins as a macular lesion that spares the peripapillary region with a higher risk of choroidal neovascularization (CNV) and poor visual outcome.
Ampiginous chorioretinopathy is characterized by multifocal plaque-like lesions scattered over the posterior pole that have overlapping features of both acute posterior multifocal placoid pigment epitheliopathy (APMPPE) and SC.
Ampiginous chorioretinopathy is characterized by multiple recurrences and progressive enlargement of serpiginous lesions over time without the spontaneous resolution typical of APMPPE.
Serpiginous Choroidopathy MANAGEMENT
Treatment of Serpiginous choroidopathy (SC) aims to stop chorioretinal inflammation especially when the advancing lesions threaten the fovea.
Systemic or periocular corticosteroids are frequently used but recurrence prevention usually requires long-term anti-inflammatory treatment with a steroid-sparing agent, such as antimetabolites (methotrexate, mycophenolate mofetil, azathioprine), cyclosporine A, or anti-TNF agents, such as adalimumab or infliximab, once tuberculosis has been ruled out.
Long-term management can be challenging and up to 25% of the eyes have a final visual acuity of less than 20/200.
The most common complication of SC is choroidal neovascularization affecting up to 35% of patients. Other reported complications are subretinal fibrosis, cystoid macular edema, branch vein occlusion, serous retinal detachment, optic disc neovascularization, and anterior uveitis.
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