Fujita Yuya, Fukui Shoichi, Suzuki Takahisa, Ishida Midori, Endo Yushiro, Tsuji Sousuke, Takatani Ayuko, Igawa Takashi, Shimizu Toshimasa, Umeda Masataka, Sumiyoshi Remi, Nishino Ayako, Koga Tomohiro, Kawashiri Shin-Ya, Iwamoto Naoki, Ichinose Kunihiro, Tamai Mami, Nakamura Hideki, Origuchi Tomoki, Abe Kuniko, Kawakami Atsushi
Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences, Japan.
Department of Community Medicine, Nagasaki University Graduate School of Biomedical Sciences, Japan.
Intern Med. 2018 Dec 1;57(23):3473-3478. doi: 10.2169/internalmedicine.1121-18. Epub 2018 Jul 6.
A 56-year-old Japanese woman with muscle weakness, increased creatine kinase and aldolase levels, and characteristic cutaneous lesions was diagnosed with anti-melanoma differentiation-associated gene 5 antibody (anti-MDA5 antibody)-positive dermatomyositis. She also had interstitial lung disease (ILD). After corticosteroid and tacrolimus combination therapy was started, bicytopenia and elevated serum ferritin and transaminase emerged. Because the bone marrow tissues were hypoplastic with hemophagocytes, she was diagnosed with concomitant autoimmune-associated hemophagocytic syndrome (HPS). Intravenous cyclophosphamide pulse therapy and plasmapheresis were performed. The laboratory findings indicated improved abnormalities, and the ILD did not progress. Anti-MDA5 antibody-positive dermatomyositis can be complicated by HPS.
一名56岁的日本女性，出现肌肉无力、肌酸激酶和醛缩酶水平升高以及特征性皮肤病变，被诊断为抗黑色素瘤分化相关基因5抗体（抗MDA5抗体）阳性皮肌炎。她还患有间质性肺病（ILD）。在开始使用皮质类固醇和他克莫司联合治疗后，出现了血细胞减少以及血清铁蛋白和转氨酶升高。由于骨髓组织发育不全并伴有噬血细胞，她被诊断为合并自身免疫相关噬血细胞综合征（HPS）。进行了静脉环磷酰胺脉冲治疗和血浆置换。实验室检查结果显示异常情况有所改善，ILD未进展。抗MDA5抗体阳性皮肌炎可并发HPS。
