Tsuji Yoshika, Iwanaga Nozomi, Adachi Anna, Tsunozaki Kinuyo, Izumi Yasumori, Moriwaki Yuji, Kurohama Kazuhiro, Ito Masahiro, Kawakami Atsushi, Migita Kiyoshi
Department of General Internal Medicine and Rheumatology, Nagasaki Medical Center, Kubara 2-1001-1, Omura 856-8562, Japan.
Nagasaki Medical Center, Kubara 2-1001-1, Omura 856-8562, Japan.
Case Rep Rheumatol. 2015;2015:163952. doi: 10.1155/2015/163952. Epub 2015 Dec 22.
We report a 64-year-old female case of intractable adult-onset Still's disease (AOSD). Initial high-dose steroid therapy combined with cyclosporin A was ineffective against macrophage-activation syndrome (MAS), which was accompanied by the systemic type of AOSD. Treatment for MAS with intravenous cyclophosphamide resulted in remission of AOSD and a reduction in the high doses of steroids. Efficacy of biologics against MAS in AOSD is unclear. Cyclophosphamide, a conventional cytotoxic agent, should be considered as one of the therapeutic options for refractory types of AOSD with MAS.
我们报告了一例64岁成年女性难治性成人斯蒂尔病(AOSD)病例。初始大剂量类固醇疗法联合环孢素A对伴有全身型AOSD的巨噬细胞活化综合征(MAS)无效。静脉注射环磷酰胺治疗MAS导致AOSD缓解以及高剂量类固醇的减量。生物制剂对AOSD中MAS的疗效尚不清楚。环磷酰胺,一种传统的细胞毒性药物,应被视为伴有MAS的难治性AOSD类型的治疗选择之一。