Ajeganova Sofia, De Becker Ann, Schots Rik
Rheumatology Department, Clinical Sciences, Vrije Universiteit Brussel, Universitair Ziekenhuis, Brussels, Belgium.
Department of Clinical Hematology, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium.
Ther Adv Musculoskelet Dis. 2020 Nov 24;12:1759720X20974858. doi: 10.1177/1759720X20974858. eCollection 2020.
Macrophage activation syndrome (MAS) is a severe, potentially fatal complication of rheumatic diseases. This case demonstrates the significant challenges and therapeutic considerations in adult-onset Still's disease (AOSD) complicated with MAS at initial presentation, which will be discussed. MAS in our patient was refractory to the first-line therapy with high-dose corticosteroids, early administration of anakinra at a standard dosage and subsequent add-on treatments with cyclosporine A, IVIG, etoposides and tocilizumab. At 2 months after presentation, the patient was still critically ill with clinical, laboratory and histological signs of an active uncontrolled MAS. Notably, adoption of anakinra at a high dosage finally induced remission. This case confirms that adjusted dosage of anakinra is an effective therapeutic strategy in a severe AOSD-related MAS. It is tempting to speculate that anakinra at a high dosage, if used earlier, would have significantly changed the course of the disease in our patient and could have led to earlier remission.
巨噬细胞活化综合征（MAS）是风湿性疾病的一种严重的、可能致命的并发症。本病例展示了成人斯蒂尔病（AOSD）初发时合并MAS所面临的重大挑战及治疗考量，将对此进行讨论。我们的患者使用大剂量皮质类固醇进行一线治疗、标准剂量早期使用阿那白滞素以及随后加用环孢素A、静脉注射免疫球蛋白、依托泊苷和托珠单抗治疗MAS均无效。就诊2个月后，患者仍病情危重，有临床、实验室及组织学证据表明MAS处于活动且未得到控制状态。值得注意的是，高剂量使用阿那白滞素最终诱导病情缓解。该病例证实，调整阿那白滞素剂量是治疗严重AOSD相关MAS的有效策略。不禁推测，若早期使用高剂量阿那白滞素，本患者的疾病进程可能会显著改变，或许能更早实现缓解。
