Onishi Yasutaka, Kawamura Tetsuji, Kagami Ryogo, Nakahara Yasuharu, Yoshiro Mochiduki
Department of Respiratory Medicine, National Hospital Organization Himeji Medical Center, Japan.
Intern Med. 2014;53(23):2701-4. doi: 10.2169/internalmedicine.53.2564. Epub 2014 Dec 1.
Immunoglobulin (Ig)-G4-related disease is a multi-organ disease that may affect the lung. We herein describe a patient with IgG4-related lung disease (IgG4-RLD) who was radiologically and pathologically diagnosed with organizing pneumonia. He was successfully treated with a combination of prednisolone (PSL) and azathioprine (AZA), and his clinical course has been uneventful since tapering off PSL. This is a rare case of IgG4-RLD manifesting as organizing pneumonia, and, to our knowledge, this is also the first case showing the effectiveness of AZA in treating IgG4-RLD.
免疫球蛋白（Ig）-G4相关疾病是一种可能累及肺部的多器官疾病。我们在此描述一名IgG4相关肺部疾病（IgG4-RLD）患者，其经影像学和病理学诊断为机化性肺炎。他接受泼尼松龙（PSL）和硫唑嘌呤（AZA）联合治疗后取得成功，自PSL减量以来，其临床病程平稳。这是一例罕见的表现为机化性肺炎的IgG4-RLD病例，据我们所知，这也是首例显示AZA治疗IgG4-RLD有效的病例。
