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

Abstract

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. Key words: Hemophagocytic syndromes;  Autoimmune diseases;  Clinical features;  Laboratory examination;  Treatment;  Prognosis