Nigrovic Peter A
Boston Children's Hospital and Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.
Arthritis Rheumatol. 2025 Apr;77(4):367-379. doi: 10.1002/art.43052. Epub 2024 Dec 12.
Macrophage activation syndrome (MAS) is a state of immune hyperactivation that can result in life-threatening multisystem end-organ dysfunction. Often termed a "cytokine storm," MAS occurs among the rheumatic diseases most typically in Still's disease but also in systemic lupus erythematosus and Kawasaki disease. MAS can also accompany infection, malignancy, and inborn errors of immunity. This review provides a practical, evidence-based guide to the understanding, recognition, and management of MAS in children and adults, with a primary focus on MAS complicating Still's disease.
巨噬细胞活化综合征（MAS）是一种免疫过度激活状态，可导致危及生命的多系统终末器官功能障碍。MAS常被称为“细胞因子风暴”，多见于风湿性疾病，最典型的是斯蒂尔病，也可见于系统性红斑狼疮和川崎病。MAS也可伴随感染、恶性肿瘤及先天性免疫缺陷。本综述为儿童和成人MAS的理解、识别及管理提供了一份实用的、基于证据的指南，主要关注并发于斯蒂尔病的MAS。
