中文摘要：

目的探讨RA继发巨噬细胞活化综合征（MAS）的临床特点，以减少误诊漏诊。提高对风湿病合并MAS的全面系统的认识。方法回顾性分析1例RA继发MAS患者的临床资料，并复习相关文献。结果本例患者为65岁老年男性，既往RA病史14年，以反复对称性多关节肿痛、晨僵14年，加重伴发热2周入院。长期使用各种药物治疗RA效果不佳，间断出现白细胞减少，使用升白细胞药物和激素治疗效果均不佳。后行骨髓穿刺及活体组织检查提示存在噬血细胞吞噬现象而明确诊断。治疗上予大剂量激素＋环孢素＋人免疫丙种球蛋白后病情明显改善；在PubMed、万方医学网数据库，以RA＋MAS为检索模式，共搜索出相关文献12篇，累计患者14例，其中男性8例，女性6例，成年人4例，未成年人10例，RA＋MAS病程最短0.5~24个月。病程中均出现发热、典型皮疹、关节炎、肝脏或脾脏肿大、血细胞的减少、转氨酶的升高、TG的升高等一系列症状和实验室检查指标的改变。结论当RA患者出现血白细胞降低且无法用其他原因解释时，应当严密地进行鉴别诊断，警惕继发MAS的发生。要认识RA炎合并MAS的风险和危害。尽早诊断，及时治疗，以最快最有效的方式挽救患者的生命。

英文摘要：

To investigate the clinical characteristics of Macrophage activation syndrome （MAS） in patients with rheumatoid arthritis （RA）, so as to reduce misdiagnosis. The objective of this paper was to improve the comprehensive and systematic understanding of rheumatoid arthritis complicated with macrophage activation syndrome.MethodsThe clinical data of one patient with macrophage activation syndrome secondary to RA were analyzed retrospectively, and the related literatures were reviewed.ResultsThe patient was a 65 year old male with ahistory of RA for 14 years. The patient presented with symmetrical multi-joint pain aggravated with stiffness for 14 years and was admitted because of aggravation for 2 weeks. He failed many drugs for the treatment of rheumatoid arthritis was ineffective accompanied with intermittent leukocytopenia. After bone marrow aspiration and biopsy, the phenomenon of phagocytosis of macrophages was clearly diagnosed. He was treatment with high dose corticosteroid＋CsA＋ human immunoglobulin and his condition wasimproved. Literature was searched in PubMed, Wan Fang medical network database, RA＋MAS. Finally, 12 related articles was yielded, and a total of 14 patients, including 8 males, 6 females. Four patients were adults and 10 were children. The shortest duration was 0.5 months, the longest was 24 months. Fever, skin rash, arthritis, enlargement of the liver or spleen, decreased of blood cells count, elevation of liver transaminase, increase of triglyceride, and a series of symptoms and laboratory parameters were observed in the course of the disease.ConclusionWhen rheumatoid arthritis patients show decreased blood leukocytes and can not be explained by other causes, the differential diagnosis should be carefully performed to rule out secondary macrophage activation syndrome. Always be awake of the risks and dangers of rheumatoid arthritis complicated with macrophage activation syndrome. Early diagnosis and timely are important to improve prognosis.