Komiya Yoji, Saito Tetsuya, Mizoguchi Fumitaka, Kohsaka Hitoshi
Department of Rheumatology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University (TMDU), Japan.
Intern Med. 2017;56(23):3237-3241. doi: 10.2169/internalmedicine.7966-16. Epub 2017 Dec 1.
A 57-year-old woman was admitted to our hospital because of a high fever, anemia, and hyperferritinemia. Since a bone marrow examination revealed hemophagocytosis, she was diagnosed with hemophagocytic syndrome (HPS). During treatment of HPS, a heliotrope rash and Gottron's sign appeared with elevated levels of serum aldolase. She also developed heart failure. She was diagnosed with dermatomyositis (DM) and associated myocarditis. Although the administration of glucocorticoids, calcineurin inhibitors, intravenous immunoglobulins, and etoposide ameliorated the clinical findings of DM and cytopenia, the fever and hyperferritinemia remained. The addition of infliximab to glucocorticoids and tacrolimus improved the fever and hyperferritinemia and enabled a reduction in the dose of prednisolone without relapse of the diseases.
一名57岁女性因高热、贫血和高铁蛋白血症入院。由于骨髓检查发现噬血细胞现象，她被诊断为噬血细胞综合征（HPS）。在HPS治疗期间，出现了向阳疹和Gottron征，血清醛缩酶水平升高。她还发生了心力衰竭。她被诊断为皮肌炎（DM）并伴有心肌炎。尽管给予糖皮质激素、钙调神经磷酸酶抑制剂、静脉注射免疫球蛋白和依托泊苷改善了DM和血细胞减少的临床表现，但发热和高铁蛋白血症仍然存在。在糖皮质激素和他克莫司基础上加用英夫利昔单抗改善了发热和高铁蛋白血症，并使得泼尼松龙剂量能够减少且疾病无复发。
