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Elise Philippakis, Valerie Touitou, Coralie Bloch-Queyrat, Aude Rigolet, Frederic Charlotte, Phuc LeHoang, Bahram Bodaghi; Ophthalmological and Histological Manifestations of IgG4-Related Disease. Invest. Ophthalmol. Vis. Sci. 2012;53(14):6245.
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IgG4-related disease is defined by both elevated serum IgG4 over 135mg/dL and histopathological features, such as lymphocytes and IgG4+ plasma cell infiltration (ratio IgG4+ plasma cells/IgG+ plasma cells over 40%), associated with typical tissue fibrosis or sclerosis. The aim of this study is to determine the ophthalmological and histocytological features of IgG4-related disease.
Patients with biopsy-proven hyper-IgG4 syndrome, seen between 2009 and 2011 in a single tertiary center, were retrospectively reviewed. Clinical features were analyzed, and compared with histological features and with patient's prognosis.
Three patients were included in the study. Mean age at diagnosis was 58 years (range:50-63 years). One patient presented with unilateral painful propotosis, associated with third and fifth cranial nerves palsies. One patient had recurrent anterior scleritis, and one patient presented with chronic conjonctival infiltration. None of them had extra-ocular involvement (FDG-PET-scan). Other inflammatory conditions were ruled out. Histocytology demonstrated lymphoplasmocytosic proliferation with overexpression of IgG4+ plasma cells and signs of fibrosis. All patients responded to corticosteroids. However one patient developped orbital fibrosis and required the adjunction of an immusuppressive drug.
IgG4 related disease is a new and rare entity, which can be challenging to diagnose when clinical features are atypical , such as isolated conjonctivitis, scleritis and orbital infiltrations. Histopathology is the gold-standard for the diagnosis. Prognosis is determined by fibrosis, sclerosis and systemic involvement(pancreatitis, retroperitoneal fibrosis and hepatitis).Treatment is not consensual, but is based on corticosteroids, associated most of the time with immunosuppressants in case of fibrosis or cortico- dependant lesions.
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