Levy Ofer, Apel Arie, Alhdor Hossam, Mizrachi Avraham, Agmon-Levin Nancy, Koren-Michowitz Maya, Amit-Vazina Mirit
Shamir Medical Center, Internal Medicine B, Rheumatology Unit, Zerifin, Israel; Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel.
Shamir Medical Center, Department of Hematology, Zerifin, Israel.
Eur J Rheumatol. 2022 Oct;9(4):217-220. doi: 10.5152/eurjrheum.2022.21064.
Macrophage activation syndrome is the most frequent life-threatening complication of adult-onset Still's disease. This is a nearly fatal case of a young patient, which has been refractory to corticoste- roids, anakinra, tocilizumab, cyclosporine A, and etoposide, but eventually responded miraculously to salvage therapy with ruxolitinib. We review recent pertinent data related to the therapeutic value of ruxolitinib for macrophage activation syndrome triggered by adult-onset Still's disease.
巨噬细胞活化综合征是成人斯蒂尔病最常见的危及生命的并发症。这是一名年轻患者的近乎致命的病例,该病例对皮质类固醇、阿那白滞素、托珠单抗、环孢素A和依托泊苷均无反应,但最终对鲁索替尼挽救治疗产生了奇迹般的反应。我们回顾了与鲁索替尼治疗成人斯蒂尔病引发的巨噬细胞活化综合征的治疗价值相关的近期相关数据。