Department of Rheumatology, The Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, Zhejiang, China.
Department of Respiratory Disease, Thoracic Disease Center, The First Affiliated Hospital, College of Medicine, Zhejiang University, Hangzhou, Zhejiang, China.
Front Immunol. 2024 Apr 26;15:1389710. doi: 10.3389/fimmu.2024.1389710. eCollection 2024.
Macrophage activation syndrome (MAS) is a rare complication of autoimmune inflammatory rheumatic diseases (AIIRD) characterized by a progressive and life-threatening condition with features including cytokine storm and hemophagocytosis. Predisposing factors are typically associated with microbial infections, genetic factors (distinct from typical genetically related hemophagocytic lymphohistiocytosis (HLH)), and inappropriate immune system overactivation. Clinical features include unremitting fever, generalized rash, hepatosplenomegaly, lymphadenopathy, anemia, worsening liver function, and neurological involvement. MAS can occur in various AIIRDs, including but not limited to systemic juvenile idiopathic arthritis (sJIA), adult-onset Still's disease (AOSD), systemic lupus erythematosus (SLE), Kawasaki disease (KD), juvenile dermatomyositis (JDM), rheumatoid arthritis (RA), and Sjögren's syndrome (SS), etc. Although progress has been made in understanding the pathogenesis and treatment of MAS, it is important to recognize the differences between different diseases and the various treatment options available. This article summarizes the cell types and cytokines involved in MAS-related diseases, the heterogeneity, and treatment options, while also comparing it to genetically related HLH.
巨噬细胞活化综合征（MAS）是一种罕见的自身免疫性炎症性风湿病（AIIRD）的并发症，其特征为细胞因子风暴和噬血细胞现象，表现为进行性和危及生命的状态。诱发因素通常与微生物感染、遗传因素（与典型的遗传性噬血细胞性淋巴组织细胞增生症（HLH）不同）和免疫系统过度激活有关。临床特征包括持续发热、全身皮疹、肝脾肿大、淋巴结病、贫血、肝功能恶化和神经系统受累。MAS 可发生于多种 AIIRD 中，包括但不限于全身幼年特发性关节炎（sJIA）、成人Still 病（AOSD）、系统性红斑狼疮（SLE）、川崎病（KD）、幼年皮肌炎（JDM）、类风湿关节炎（RA）和干燥综合征（SS）等。尽管在理解 MAS 的发病机制和治疗方面取得了进展，但认识不同疾病之间的差异以及可用的各种治疗选择仍然很重要。本文总结了与 MAS 相关疾病的细胞类型和细胞因子、异质性和治疗选择，并将其与遗传性 HLH 进行了比较。
