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Serpiginous choroidopathy (SC) is a rare disease inducing a permanent loss of vision, caused by a progressive destruction of the retinal pigment epithelium and choriocapillaris. Until now, no aetiology or predisposing factors have been reported. SC, usually, affects both eyes and occurs in patients between the fourth and sixth decade, without any sex or race predilection. Clinically, deep cream-coloured lesions develop in the peripapillary region and then along the retinal vessels, centrifugally, inducing an atrophy of the retina. Other lesions may develop, isolated, in the posterior segment. The anterior segment is typically quiet; nevertheless, a mild anterior uveitis and/or vitritis have been observed. The course of the disease results in successive attacks and recurrences inducing permanent retinal atrophic changes and subsequently an irreversible loss of vision. Choroidal neovascularisation may occasionally develop. No specific diagnostic tests are available such that the diagnosis of SC is mostly clinical.1
A 30 year old Indian man presented with a history of painless progressive visual loss affecting the right eye. No other ophthalmological or systemic complaints were present. His medical history was unremarkable.
Ophthalmological examination revealed a visual acuity of 20/50 in the right eye and 20/20 in the left eye without a correction in both eyes. Anterior segment examination revealed a mild inflammation with fine keratic precipitates on the inferior part of the right corneal endothelium. Intra-ocular pressure was 10 mm Hg in both eyes. Fundus examination of the right eye disclosed a moderate vitreous inflammation (cells: +) and multiple deep cream choroidal lesions around the optic disc and along the superior and inferior retinal (temporal and nasal) vessels (fig 1). Our differential diagnosis was a white dot syndrome (APMPPE, SC), an infection (tuberculosis), or a sarcoidosis. We decided to hospitalise the patient.
A clinical examination revealed an erythrocyte sedimentation rate of 8 mm in the first hour (normal range 1–12), and a normal white blood count. Immunoglobulin electrophoresis, quantitative immunoglobulin levels, CD4-CD8 lymphocytes count, C3-C4 and CH50 examination were within the normal range. Tests for connective tissue disorders were negative and serum angiotensin converting enzyme was discreetly elevated (74 U/l, normal range = 18–55) with a normal lysozyme level. Infectious serologies (toxoplasmosis, Borrelia burgdorferi, Treponema pallidum, HIV, herpesvirus, Leptospirosis, Bartonella, rickettsiosis, brucellosis) were within the normal limits. An anterior chamber tap (polymerase chain reaction for herpes simplex virus (HSV) 1, HSV2, varicella zoster virus, cytomegatovirus, Epstein-Barr virus, toxoplasmosis, Mycobacteriumtuberculosis) was negative. A lumbar puncture was normal (proteins 0.31 g/l, white cells 3×106/l, lymphocytes 74%), without oligoclonal bands on electrophoresis. PPD skin test was positive (15 mm) but chest x ray was normal. We have to consider that the patient has had a BCG vaccine in his childhood. The patient was HLA-B27 and A-29 negative but HLA B-7 positive. The initial clinical examination was completed by a neurological and a dermatological examination which were normal. A magnetic resonance image cerebral scan was normal.
The patient was given a course of methylprednisolone intravenously (4×250 mg/day for 5 days) followed by oral prednisone (1 mg/kg) at tapering doses, and aciclovir (3×10 mg/kg), intravenously for 10 days. We covered the patient with rifampicin, isoniazide, ethambutol, pyrazinamide, and B6 vitamin. Topical steroids and mydriatics were administered.
A regression of the inflammation in the right eye was noted as well as a “cicatrisation” of the choroidal lesions, which appeared as multiple geographical areas of atrophy of both the retina and pigmentary epithelium between areas of normal retina. Our suspected diagnosis was a SC.
After 3 weeks, the patient developed the same lesions in the left eye with an occlusion of the superior temporal vein (fig 2). At that time the patient was on prednisone 40 mg/day and anti-TB treatment. A complete clinical examination was done again, but still all results were within the normal limits. The same treament was introduced (methylprednisolone, intravenous aciclovir).
As the relapse occured under steroid therapy (prednisone 40 mg/day), the administration of an immunosuppressive drug was discussed. The patient was given mycophenolate mofetyl (Cellcept, 2 g/day) and oral prednisone for 1 year, at tapering doses. Anti-TB treatment was continued too. No secondary effects were noted.
We followed the patient during 12 months; his visual acuity returned to 20/20, without a correction, in both eyes. The anterior segment was normal. Posterior segment examination disclosed permanent geographic chorioretinal atrophic lesions along the vessels in both eyes, confirming the diagnosis of SC.
SC induces a progressive loss of the retinal pigment epithelium and choriocapillaris. The cause of this disorder is still under investigation but some studies suggest that an inflammatory or a vascular factor are involved in the pathogenesis.2 Histopathological studies have shown the presence of extensive lymphocyte choroidal infiltrates but in eyes chronically affected.3 The distribution of the lesions and their angiographic features (fluorescein and green indocyanine) may suggest a choroidal occlusion.4 Genetic studies demonstrated an increased frequency of HLA-B7 in this affection5; our patient was positive. SC affects not only white people but also oriental and blacks people. There is no sex predilection and the patients are middle aged when the diagnosis is made. The patient described in this report was 30 year old which is uncharacteristic. The aetiology remains unknown although recently tubercular serpiginous-like choroiditis has been reported.6
Clinically, inflammatory signs may be noted both in segments (anterior uveitis, vitritis) along with the classic whitish choroidal lesions in the acute stage. The most frequent ocular complication of SC is subretinal neovascularisation which affects 13% to 20% of the eyes. Retinal vasculitis is also observed.
Gupta et al reported a case of SC with a branch vein occlusion in the acute phase.6 Our patient developed a vein occlusion while under treatment (steroids, aciclovir, and anti-TB therapy) which is rare and was never reported to our knowledge. Haemorrhages are sometimes observed in inflammatory diseases (Behçet’s syndrome, sarcoidosis) or in infectious posterior uveitis (syphilis, viral infections, toxoplasmosis7).
This case demonstrates that SC can affect young patients and that HLA-B7 can be found in Indian patients with SC. The clinical features (vascular occlusion in this case) and the development of new lesions while under treatment let us suspect that the cause of this disease is still not clear.