Heidari Pegah, Verdijk Robert M, van den Bosch W A, Paridaens Dion
The Rotterdam Eye Hospital, Oculoplastic & Orbital Service , Rotterdam , the Netherlands .
Orbit. 2014 Oct;33(5):388-91. doi: 10.3109/01676830.2014.904380. Epub 2014 Jun 9.
A 38-year-old female patient presented with a painful swelling in the lateral part of the upper eyelid, a diffuse scleritis and slight hypoglobus of the right eye. An orbital biopsy showed a fibrotic idiopathic orbital inflammation (IOI) with, on immunohistochemical staining, an increased number of IgG4-positive plasma cells scored as >200 per high-power field, with IgG4/IgG ratio >0.50, indicating orbital IgG4 related autoimmune disease. On treatment with oral prednisone and azathioprine the symptoms resolved within 6 months. Twenty years prior, the patient had been diagnosed with an IOI of at the same side, for which at that time a biopsy had been taken similarly. Reclassification of the previous biopsy specimen with immunohistological staining also showed evidence of orbital IgG4 related disease. To our knowledge this is the first report of a biopsy-proven unilateral IgG4-related orbitopathy that recurred after 20 years.
一名38岁女性患者，上睑外侧出现疼痛性肿胀，伴有弥漫性巩膜炎及右眼轻度眼球内陷。眼眶活检显示为纤维化特发性眼眶炎症（IOI），免疫组化染色显示，每高倍视野IgG4阳性浆细胞数量增加，>200个，IgG4/IgG比率>0.50，提示眼眶IgG4相关自身免疫性疾病。经口服泼尼松和硫唑嘌呤治疗后，症状在6个月内缓解。20年前，该患者曾被诊断为同侧IOI，当时同样进行了活检。对之前的活检标本进行免疫组织化学染色重新分类，也显示出眼眶IgG4相关疾病的证据。据我们所知，这是首例经活检证实的单侧IgG4相关眼眶病20年后复发的报告。
