Dall'Ara F, Cavazzana I, Frassi M, Taraborelli M, Fredi M, Franceschini F, Andreoli L, Rossi M, Cattaneo C, Tincani A, Airò P
Rheumatology and Clinical Immunology, Department of Clinical and Experimental Sciences, Spedali Civili and University of Brescia.
Reumatismo. 2018 Jul 6;70(2):100-105. doi: 10.4081/reumatismo.2018.1023.
The aim was to describe the macrophage activation syndrome (MAS), a life-threatening syndrome characterized by excessive immune activation that can be triggered by conditions affecting immune homeostasis, in a cohort of adult Italian patients with systemic lupus erythematosus (SLE). This was a monocentric retrospective evaluation. The utility of the H-score, developed to estimate the individual risk of having reactive MAS in adult patients, was assessed. Among 511 patients with SLE, 7 cases (1.4%) of MAS (all females) were identified and their medical records reviewed. In all cases, MAS was simultaneous to the onset of SLE. All patients had fever, lymphadenopathy, hematological involvement, and high titer of anti-dsDNA antibodies. Workup for infections and malignancies was negative. In all cases, the H-score was higher than the cut-off suggested for the classification of reactive MAS. All cases required hospital admission, and 2 patients were admitted to the intensive care unit. Most patients were treated successfully with high doses of corticosteroids and with immunosuppressive drugs, whereas the full therapeutic regimen developed for primary hemophagocytic lymphohistiocytosis HLH was used only in one case. No death from MAS was observed. MAS is a rare and severe disorder that complicated the onset of SLE in our cohort. The H-score may be useful in the classification of these patients.
目的是描述巨噬细胞活化综合征（MAS），这是一种危及生命的综合征，其特征为过度免疫激活，可由影响免疫稳态的状况引发，研究对象为一组成年意大利系统性红斑狼疮（SLE）患者。这是一项单中心回顾性评估。评估了用于估计成年患者发生反应性MAS个体风险的H评分的效用。在511例SLE患者中，识别出7例（1.4%）MAS患者（均为女性）并对其病历进行了回顾。所有病例中，MAS均与SLE起病同时发生。所有患者均有发热、淋巴结病、血液系统受累及高滴度抗双链DNA抗体。感染和恶性肿瘤检查均为阴性。所有病例中，H评分均高于反应性MAS分类建议的临界值。所有病例均需住院，2例患者入住重症监护病房。大多数患者接受大剂量糖皮质激素和免疫抑制药物治疗成功，而仅1例患者使用了为原发性噬血细胞性淋巴组织细胞增生症（HLH）制定的完整治疗方案。未观察到因MAS死亡的病例。MAS是一种罕见且严重的疾病，在我们的队列中使SLE起病复杂化。H评分可能有助于这些患者的分类。
