Liu Ailing, Zhang Qianwen, Liu Bo, Xu Ning, Li Aijun
Department of Pulmonary Diseases, Weihai Municipal Hospital, Weihai, Shandong, P.R. China.
Medicine (Baltimore). 2017 Dec;96(50):e9211. doi: 10.1097/MD.0000000000009211.
Immunoglobulin (Ig) G4-related disease (IgG4-RD) is a rare and chronic progressive autoimmune disease. It is a novel clinical entity characterized by elevated serum IgG4 concentration and tissue infiltration by IgG4 plasma cells. IgG4-related lung disease (IgG4-RLD) has been described as interstitial pneumonia and inflammatory pseudotumor, with various abnormal radiographic patterns. We report a case of IgG4-related lung disease with bilateral diffuse infiltration.
A 65-year-old woman was admitted to our hospital because of cough, sputum, and fever. Chest computed tomography (CT) revealed multifocal areas of consolidations, nodules, and ground glass opacities in both lungs. She still had fever after anti-infective therapy.
Bronchial bronchoscopy and percutaneous lung biopsy were performed, resulted in a pathological diagnosis of IgG4-RLD.
The patient was started on oral prednisolone 30 mg/day for 28 days, and then was gradually tapered.
After one week treatment, the temperature was stable, CT showed significant improvement in the areas of consolidations and nodules.
It is a typical case of IgG4-RLD. This case indicated that suggestive evidence, radiological appearances, serum tests, pathological characteristics, and classic therapy IgG4-RLD. It is a rare disease that needs our more attention in future.
免疫球蛋白G4相关疾病（IgG4-RD）是一种罕见的慢性进行性自身免疫性疾病。它是一种新型临床实体，其特征为血清IgG4浓度升高以及IgG4浆细胞浸润组织。IgG4相关肺部疾病（IgG4-RLD）已被描述为间质性肺炎和炎性假瘤，具有多种异常影像学表现。我们报告一例双侧弥漫性浸润的IgG4相关肺部疾病病例。
一名65岁女性因咳嗽、咳痰和发热入院。胸部计算机断层扫描（CT）显示双肺多发实变、结节及磨玻璃影区域。抗感染治疗后仍有发热。
进行了支气管镜检查和经皮肺活检，病理诊断为IgG4-RLD。
患者开始口服泼尼松龙30mg/天，持续28天，然后逐渐减量。
治疗一周后，体温稳定，CT显示实变和结节区域有明显改善。
这是一例典型的IgG4-RLD病例。该病例表明了IgG4-RLD的提示性证据、影像学表现、血清学检查、病理特征及经典治疗方法。这是一种罕见疾病，未来需要我们更多关注。
