Lee Ju Ho, Ha You-Jung, Kang Eun Ha, Chang Sung Hae, Lee Yun Jong
Division of Rheumatology, Department of Internal Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
Division of Rheumatology, Department of Internal Medicine, Soonchunhyang University College of Medicine, Cheonan, Korea.
J Rheum Dis. 2022 Apr 1;29(2):123-128. doi: 10.4078/jrd.2022.29.2.123.
Macrophage activation syndrome (MAS) is a fatal complication of adult-onset Still's disease (AOSD). Although anti-cytokine agents have been recommended for refractory AOSD or complicated with MAS, MAS cases have been rarely reported during anti-cytokine treatment. Herein, we describe the first AOSD case complicated with MAS during the treatment with tocilizumab in Korea. Two years after tocilizumab maintenance therapy, high fever and hypertransaminasemia recurred. MAS was diagnosed based on hyperferritinemia, elevated soluble IL-2 receptor levels, and the presence of hemophagocytic histiocytes in the bone marrow. However, she had normal white blood cell counts and acute phase reactant levels. High-dose glucocorticoid and anakinra therapies were not effective, but her disease improved with etoposide. This case shows that tocilizumab may not prevent MAS development and can modify clinical features making it challenging to diagnose. Cytotoxic therapy such as etoposide may be required in MAS cases that develop during anti-cytokine therapy.
巨噬细胞活化综合征（MAS）是成人斯蒂尔病（AOSD）的一种致命并发症。尽管抗细胞因子药物已被推荐用于难治性AOSD或合并MAS的患者，但在抗细胞因子治疗期间很少有MAS病例的报道。在此，我们描述了韩国首例在使用托珠单抗治疗期间并发MAS的AOSD病例。在托珠单抗维持治疗两年后，高热和高转氨酶血症复发。根据高铁蛋白血症、可溶性白细胞介素-2受体水平升高以及骨髓中存在噬血细胞组织细胞，诊断为MAS。然而，她的白细胞计数和急性期反应物水平正常。大剂量糖皮质激素和阿那白滞素治疗无效，但她的病情在使用依托泊苷后有所改善。该病例表明，托珠单抗可能无法预防MAS的发生，且可能改变临床特征，使其诊断具有挑战性。在抗细胞因子治疗期间发生的MAS病例可能需要使用依托泊苷等细胞毒性疗法。
