Philippakis Elise, Cassoux Nathalie, Charlotte Frédéric, LeHoang Phuc, Bodaghi Bahram, Bloch-Queyrat Coralie, Touitou Valérie
Ophthalmology Department, Pitie-Salpetriere Hospital , Paris , France .
Ocul Immunol Inflamm. 2015 Apr;23(2):168-72. doi: 10.3109/09273948.2014.985384. Epub 2014 Dec 30.
To report atypical ophthalmologic manifestations and complications of IgG4-related disease (IgG4-RD).
Patients with isolated ophthalmologic involvement of IgG4-RD other than lacrimal or orbital infiltration seen between 2009 and 2011 in a single tertiary center were retrospectively reviewed and their clinical and histological features, treatment, and prognosis were studied.
Two patients (mean age 56.5 years) were included. One patient presented with recurrent anterior and posterior scleritis, and one patient had chronic conjunctival infiltration. Histopathology demonstrated lymphoplasmacytic proliferation with overexpression of IgG4(+) plasma cells. Both patients initially responded to a high dose of oral corticosteroids (1 mg/kg/d). However, one patient required the adjunction of methotrexate and one patient developed an intra-epithelial conjunctival carcinoma on the site of the initial lesion.
Patients with atypical presentation of IgG4-RD, such as chronic conjunctival infiltration or scleritis, can suffer from considerable diagnostic delay leading to fibrosis or malignancy development. We report the first case of conjunctival carcinoma in a patient with IgG4-RD.
报告IgG4相关性疾病（IgG4-RD）的非典型眼科表现及并发症。
回顾性分析2009年至2011年在某单一三级中心就诊的、除泪腺或眼眶浸润外孤立性眼科受累的IgG4-RD患者，研究其临床和组织学特征、治疗及预后。
纳入2例患者（平均年龄56.5岁）。1例患者表现为复发性前巩膜炎和后巩膜炎，另1例患者有慢性结膜浸润。组织病理学显示淋巴细胞和浆细胞增生，IgG4(+)浆细胞过表达。2例患者最初对高剂量口服糖皮质激素（1mg/kg/d）有反应。然而，1例患者需要加用甲氨蝶呤，另1例患者在初始病变部位发生上皮内结膜癌。
IgG4-RD非典型表现（如慢性结膜浸润或巩膜炎）的患者可能会有相当长的诊断延迟，导致纤维化或恶性肿瘤发生。我们报告了首例IgG4-RD患者并发结膜癌的病例。
