自身免疫病相关噬血细胞综合征29例临床分析

Clinical analysis of 29 patients with autoimmune disease-associated hemophagocytic syndromes

目的 提高对自身免疫病相关噬血细胞综合征（AAHS）的认识和诊疗水平.方法 对2004年4月至2014年4月间确诊为AAHS患者的临床表现、实验室检查、诊断、治疗方案及临床转归进行回顾性综合分析.结果 29例AAHS患者中女22例,男7例,中位年龄31岁（16～70岁）.原发病为成年人斯蒂尔病17例,干燥综合征5例,系统性红斑狼疮4例,多发性肌炎1例,原发性胆汁性肝硬化1例,未分化结缔组织病1例.19例患者在自身免疫病（AID）治疗过程中继发噬血细胞综合征（HPS）,10例患者AID与HPS同时发生.AAHS的主要临床表现以发热（100.0％,29/29）、呼吸系统症状（79.3％,23/29）、脾大（72.4％,21/29）、皮疹（65.5％,19/29）、关节疼痛（62.1％,18/29）最为常见.29例AAHS患者治疗8周内,13例临床有效,8例达到临床缓解.患者1、3、12个月总生存率分别为93.1％、82.3％、72.0％.结论 AAHS患者的原发病以成年人斯蒂尔病、干燥综合征、系统性红斑狼疮为主,HPS与AID可同时发生,也可以在AID治疗过程中继发.当HPS患者伴有皮疹及关节疼痛时需考虑到AID的可能.与其他类型的HPS患者相比,AAHS的预后相对较好.

Objective To analyze the clinical characters,treatment and prognosis of autoimmune disease-associated hemophagocytic syndromes （AAHS）.Methods A retrospective study was carried out to analyze the clinical features,laboratory tests,diagnosis,treatments and clinical outcomes of 29 patients with AAHS from Apr.2004 to Apr.2014.Results Among the 29 cases,22 were female while the other 7 were male.The median age was 31 years old （16-70 years old）.The initiating diseases consisted of 17 adult onset still＇s disease,5 sicca syndrome,4 systemic lupus erythematosus,1 polymyositis,primary,1 biliary cirrhosis,1 undifferentiated connective tissue disease.19 patients of them were diagnosed as hemophagocytic syndromes （HPS） in the treatment of autoimmune disease while the other 10 patients were diagnosed as autoimmune disease and HPS at the same time.The most common clinical features were persistent fever （100.0 ％,29/29）,respiratory symptom （79.3 ％,23/29）,splenomegaly （72.4 ％,21/29）,rash （65.5 ％,19/29）,arthralgia （62.1％,28/29）.Among the 29 patients,13 patients had acquired clinical effective and 8 patients had acquired clinical alleviate within 8 weeks treatment.Among the 29 cases,1-month,3-month and 1-year overall survival rates were 93.1％,82.3 ％ and 72.0 ％ respectively.Conclusions The initiating diseases of AAHS mainly consist of adult onset still＇s disease,sicca syndrome and systemic lupus erythematosus.HPS can not only onset at the same time with autoimmune disease,but also onset in the treatment of autoimmune disease.Autoimmune disease should be considered when HPS patients complicated by rash and arthralgia.Compared with other types of HPS patients,the prognosis of AAHS is better relatively.