Carbone Teresa, Azêdo Montes Ricardo, Andrade Beatriz, Lanzieri Pedro, Mocarzel Luis
Department of Internal Medicine, Hospital Universitário Antônio Pedro (HUAP), Universidade Federal Fluminense (UFF), Rua Marquês de Paraná 303, 7° andar, Centro, 24033-900 Niterói, RJ, Brazil.
Department of Rheumatology, Hospital Universitário Antônio Pedro (HUAP), Universidade Federal Fluminense (UFF), Rua Marquês de Paraná 303, 7° andar, Centro, 24033-900 Niterói, RJ, Brazil.
Case Rep Rheumatol. 2015;2015:324365. doi: 10.1155/2015/324365. Epub 2015 Mar 9.
IgG4-related disease (IgG4-RD) encompasses a group of fibroinflammatory conditions recognized in recent times. The main clinical features include variable degrees of tissue fibrosis, tumorlike expansions, perivascular lymphocytic infiltration rich in IgG4-positive plasma cells, and elevated serum IgG4. A case has been reported of an elderly patient with an unexplained unilateral exophthalmia; biopsy was performed and revealed lymphocytic infiltration, suggesting IgG4-RD. High serum levels of IgG4, in association with a good response to steroid therapy and to the exclusion of other diagnoses, confirmed the hypothesis of orbital pseudotumor by IgG4-RD.
IgG4相关性疾病（IgG4-RD）是近年来发现的一组纤维炎性疾病。其主要临床特征包括不同程度的组织纤维化、肿瘤样扩张、富含IgG4阳性浆细胞的血管周围淋巴细胞浸润以及血清IgG4升高。有报道称一名老年患者出现不明原因的单侧眼球突出；进行活检后发现淋巴细胞浸润，提示为IgG4-RD。高血清IgG4水平，结合对类固醇治疗的良好反应以及排除其他诊断，证实了IgG4-RD导致眼眶假瘤的推测。
