Clinical and serologic features of primary Sjögren's syndrome concomitant with autoimmune hemolytic anemia: a large-scale cross-sectional study

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• 作者：Wen Wen ; Yanying Liu ; Chuchu Zhao ; Xiaolin Sun ; Chunfang Zhang…
• 关键词：Anemia ; Autoimmunity ; Cytopenia ; Primary biliary cirrhosis ; Sjögren’s syndrome
• 刊名：Clinical Rheumatology
• 出版年：2015
• 出版时间：November 2015
• 年：2015
• 卷：34
• 期：11
• 页码：1877-1884
• 全文大小：279 KB
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• 作者单位：Wen Wen (1)
  Yanying Liu (1)
  Chuchu Zhao (1)
  Xiaolin Sun (1)
  Chunfang Zhang (2)
  Zhanguo Li (1)
  
  1. Department of Rheumatology and Immunology, Clinical Immunology Center, Peking University People’s Hospital and Beijing Key Laboratory for Rheumatism Mechanism and Immune Diagnosis (BZ0135), 11, Xizhimen South Street, Beijing, 100044, China
  2. Department of Clinical Epidemiology and Biostatistics, Peking University People’s Hospital, Beijing, China
  
• 刊物类别：Medicine
• 刊物主题：Medicine & Public Health
  Rheumatology
  
• 出版者：Springer London
• ISSN：1434-9949

文摘

Autoimmune hemolytic anemia (AIHA) is an uncommon but clinically significant disorder in primary Sjögren’s syndrome (pSS). Among 565 pSS patients hospitalized in Peking University People’s Hospital from January 2000 to March 2013, 16 patients were diagnosed with AIHA (2.8 % prevalence). AIHA presented at the onset of pSS without overt sicca symptoms in 3 of the 16 patients. Primary biliary cirrhosis (PBC) was more prevalent in the patients with SS-AIHA than in those without (p = 0.007). Edema, fever, and liver involvement occurred significantly more frequently in pSS patients with AIHA than those without AIHA (p = 0.035, p = 0.029, p = 0.024, respectively). The pSS patients with AIHA were more vulnerable to leukopenia and thrombocytopenia than those without AIHA (p = 0.004 and p = 0.001, respectively). Additionally, the levels of complement component 3 (C3) and complement component 4 (C4) were significantly lower in the SS-AIHA group (p = 0.008 and p = 0.037, respectively). Taken together, our results indicate that pSS should be considered in the differential diagnosis of AIHA, even in the absence of sicca symptoms. Among pSS patients, the existence of PBC, cytopenia, or hypocomplementemia suggests a higher risk of suffering from AIHA.