结缔组织病相关噬血细胞综合征的诊治

Diagnosis and treatment of hemophagocytic syndrome associated with connective tissue disease

巨噬细胞活化综合征(MAS)是一种严重的、可能危及生命的风湿性疾病并发症,早期识别、早期诊断和及时恰当的治疗是改善预后的关键。目前MAS尚无公认的诊断标准,几种临床常用的诊断标准需要更多的研究进一步验证。应重视血清铁蛋白滴度、血清铁蛋白/ESR比例等血清标志物的监测,以提高对MAS的警觉。MAS治疗应该考虑到原发病及诱发因素(尤其是感染),进行个体化治疗。MAS的传统治疗方案为大剂量激素联合免疫抑制剂,对于难治性MAS可尝试新型靶向药物治疗。

Macrophage activation syndrome(MAS)is a severe,possibly life-threatening complication of rheumatic diseases.Early identification and diagnosis,as well as timely and appropriate treatment are essential to improve prognosis.At present,there are no unified diagnostic criteria for MAS.Most clinical diagnostic criteria commonly used now are needed to be further verified by more studies.It should pay attention to the monitoring of serum markers such as ferritin titer and ferritin/ESR ratio to be alert to MAS.Treatment of MAS should be individualized,taking into account the activity of rheumatic disease and predisposing factors(especially infection).The traditional treatment of MAS is high-dose glucocorticoid combined with immunosuppressive agents,and novel targeted drugs can be tried for refractory MAS.