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T. Grenet, C. Chapelon, J.-C. Piette, G. Ducos, N. Cassoux, C. Fardeau, P. LeHoang, B. Bodaghi; Diagnosis and Management of Biopsy-Proven Ocular Sarcoidosis in France. Invest. Ophthalmol. Vis. Sci. 2007;48(13):5155.
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To describe clinical features and paraclinical findings in patients managed for biopsy-proven ocular sarcoidosis at Pitié-Salpêtrière Hospital, Paris, France.
Clinical presentation, angiographic features, laboratory investigations, treatment strategies and visual outcome of patients managed between 2000 and 2005 for biopsy-proven ocular sarcoidosis at Pitie-Salpetriere Hospital were retrospectively reviewed.
Among 3740 patients referred for the management of a first episode of uveitis, we identified 210 patients (5.6%) with presumed ocular sarcoidosis and 53 patients (20 M and 33 F) with biopsy-proven ocular sarcoidosis (1.4%). Mean age at diagnosis was 41.8 years (range 11-80 y). Most of patients were Caucasian (55%). Granulomatous panuveitis was present in 59% of cases followed by posterior uveitis (24%), anterior or intermediate uveitis (10%) and other forms of ocular inflammation in 7% of cases. Abnormal laboratory findings included : serum angiotensin converting enzyme (79.5%) and lysozyme (88.6%), chest CT-scan (83.3%), bronchoalveolar lavage (77.8%), gallium-scan (72%) and negative PPD (80%). Extraocular manifestations of sarcoidosis were diagnosed in 84.9% of patients, including pulmonary and neurological involvement in most of the cases. Histological confirmation was performed based on bronchoalveolar biopsy (40%); salivary gland biopsy (24%); cutaneous biopsy (9%) or other types (27%). Corticosteroids were used in 84.5% of cases and immunosuppressors were mandatory in 41% of cases. Methotrexate (40.9%) and azathioprine (22.7%) were the most commonly used drugs. Ocular complications occurred in 37.7% of cases, including macular edema (24.5%), choroidal neovascularisation (3.7%) and inflammatory retinal vein occlusion with secondary neovascular glaucoma (3.1%). Final visual acuity was improved or stabilized in most of patients (86%) with a mean follow-up of 36.3 months (range 1-204 months)Discussion: Biopsy-proven ocular sarcoidosis remains a rare cause of chronic uveitis in France. Corticosteroids remain the mainstay of treatment with a good visual outcome in most of cases. Despite the absence of histological evidences, the management of presumed ocular sarcoidosis is based on a similar strategy.
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