Department of Hematology, Nephrology, and Rheumatology, Akita University Graduate School of Medicine, Akita, Japan.
Department of Hematology, Akita Kousei Medical Center, Akita, Japan.
Mod Rheumatol Case Rep. 2022 Jun 23;6(2):278-281. doi: 10.1093/mrcr/rxac025.
Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) involves multiple organs, including the lungs and central nervous system. Lung lesions are frequently reported as mass lesions or non-specific interstitial pneumonia, whereas organising pneumonia (OP) due to IgG4-RD is rare. Furthermore, limited information is currently available on hypertrophic pachymeningitis (HP). We herein report a case of IgG4-RD complicated with OP and HP. The diagnosis was confirmed based on the serum concentration of IgG4 and the results of salivary gland and transbronchial lung biopsies. HP did not respond to steroid monotherapy and was also resistant to rituximab and intravenous cyclophosphamide; however, the combination therapy of methotrexate and dexamethasone was effective.
免疫球蛋白 G4（IgG4）相关疾病（IgG4-RD）涉及多个器官，包括肺部和中枢神经系统。肺部病变常表现为肿块病变或非特异性间质性肺炎，而 IgG4-RD 导致的机化性肺炎（OP）则较为罕见。此外，目前有关肥厚性硬脑膜炎（HP）的信息有限。本文报告了一例 IgG4-RD 合并 OP 和 HP 的病例。该诊断基于 IgG4 血清浓度以及唾液腺和经支气管肺活检结果而确认。HP 对类固醇单药治疗无反应，也对利妥昔单抗和静脉环磷酰胺耐药；然而，甲氨蝶呤和地塞米松联合治疗有效。
