IgG4 相关疾病 (IgG4 RD) 是一种免疫介导的纤维炎症性疾病，伴有 IgG4 浆细胞的组织浸润。它引起假瘤、肿瘤和广泛的临床表现。报告一组智利 IgG4 RD 患者的临床、实验室、组织病理学和治疗特征。回顾 52 名 18 至 76 岁 IgG4 RD 患者的病历在六个医疗中心进行了观察。44 名患者中有 18 名 (41%) 发现血清 IgG4 水平升高 (> 135 mg/dl)。 46 名患者经组织学证实该疾病。最常见的受累部位是肺、眼睛和肾脏。 18 名患者 (35%) 仅受累一种器官，34 名患者 (65%) 受累两个器官，13 名患者 (25%) 受累三个或更多器官。两个器官受累在男性中更为常见 (p < 0.05)。在仅一种器官受累的患者中，最常见的部位是眼眶和脑膜。所有患有肾脏或肺部疾病的患者都有多器官受累。所有患者均接受皮质类固醇治疗，67% 为合成免疫抑制剂，16% 为利妥昔单抗。ER-IgG4 可影响任何组织。多器官受累在该系列中更为常见，尤其是肺、眼睛和肾脏。对类固醇的良好反应是该疾病的特征，但复发率很高，需要额外的免疫抑制。

IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations.To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD.Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers.Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab.ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.