一名患有3型自身免疫性多腺体综合征的患者，在诊断自身免疫性肝炎8年后发生了系统性红斑狼疮。

A Patient with Type 3 Autoimmune Polyglandular Syndrome who Developed Systemic Lupus Erythematosus 8 years after the Diagnosis of Autoimmune Hepatitis.

作者信息

Mifune-Morioka Tomoyo, Uchida Haruhito A, Fukushima Kazuhiko, Watanabe Mayu, Ouchi Chihiro, Mise Koki, Kawakita Chieko, Kano Yuzuki, Onishi Akifumi, Toma Kishio, Eguchi Jun, Wada Nozomu, Ikeda Fusao, Sasaki Erika, Suganami Yu, Kishida Masayuki, Sugiyama Hitoshi, Okada Hiroyuki, Wada Jun

机构信息

Department of Nephrology, Rheumatology, Endocrinology and Metabolism, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.

Department of Chronic Kidney Disease and Cardiovascular Disease, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan.

出版信息

Acta Med Okayama. 2019 Aug;73(4):367-372. doi: 10.18926/AMO/56940.

DOI:10.18926/AMO/56940

PMID:31439961

Abstract

Eight years prior to her present admission, a 61-year-old Japanese woman was diagnosed with autoimmune hepatitis, slowly progressive insulin-dependent diabetes mellitus, and chronic thyroiditis; she had been treated with oral prednisolone (PSL). After she suddenly discontinued PSL, she newly developed systemic lupus erythematosus. A combination therapy of oral PSL and intravenous cyclophosphamide resulted in remission. She was finally diagnosed with autoimmune polyglandular syndrome (APS) type 3 (3A ,3B, 3D), complicated with four different autoimmune diseases. Since patients with type 3 APS may present many manifestations over a long period of time, they should be carefully monitored.

摘要

在本次入院前八年，一名61岁的日本女性被诊断出患有自身免疫性肝炎、缓慢进展的胰岛素依赖型糖尿病和慢性甲状腺炎；她一直接受口服泼尼松龙（PSL）治疗。在她突然停用PSL后，她新患上了系统性红斑狼疮。口服PSL和静脉注射环磷酰胺的联合治疗使病情缓解。她最终被诊断为3型自身免疫性多腺体综合征（APS）（3A、3B、3D型），并发四种不同的自身免疫性疾病。由于3型APS患者可能在很长一段时间内出现多种表现，因此应对他们进行仔细监测。