Sane Mona, Chelnis James, Kozielski Rafal, Fasiuddin Airaj
Ross Eye Institute, State University of New York at Buffalo, Buffalo, New York.
J AAPOS. 2013 Oct;17(5):548-50. doi: 10.1016/j.jaapos.2013.05.017.
Immunoglobulin G4-related sclerosing disease is a rare but potentially debilitating cause of orbital inflammation, with a predilection for older males. We report the case of a 12-year-old African girl with immunoglobulin G4-related sclerosing disease, including possible extraorbital involvement. Because of an escalating severity of illness leading to oculomotor nerve palsy and cavernous sinus thrombosis that was resistant to steroids, systemic immunosuppressive therapy with rituximab was used to achieve disease remission. The diagnosis was histologically confirmed with a tissue biopsy.
免疫球蛋白G4相关性硬化性疾病是一种罕见但可能导致机体衰弱的眼眶炎症病因，好发于老年男性。我们报告一例12岁非洲女孩患免疫球蛋白G4相关性硬化性疾病的病例，包括可能存在眶外受累情况。由于病情不断加重，导致动眼神经麻痹和海绵窦血栓形成，且对类固醇治疗无效，遂使用利妥昔单抗进行全身免疫抑制治疗以实现疾病缓解。通过组织活检在组织学上确诊了该疾病。
