Clinical and serologic features of primary Sjogren's syndrome concomitant with autoimmune hemolytic anemia: a large-scale cross-sectional study
Clinical and serologic features of primary Sjogren's syndrome concomitant with autoimmune hemolytic anemia: a large-scale cross-sectional study
复制标题
原发性干燥综合征合并自身免疫性溶血性贫血的临床和血清学特征：一项大规模横断面研究。
DOI：
10.1007/s10067-015-3081-0
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发表时间：
2015-11-01
影响因子：
3.4
通讯作者：
Li, Zhanguo
中科院分区：
文献类型：
--
作者：
Wen, Wen;Liu, Yanying;Li, Zhanguo
自身免疫性溶血性贫血（AIHA）是原发性干燥综合征（pSS）中一种不常见但具有临床意义的疾病。2000年1月至2013年3月在北京大学人民医院住院的565例pSS患者中，16例患者被诊断为AIHA（患病率为2.8%）。16例患者中3例在pSS发病时出现AIHA，无明显干燥症状。原发性胆汁性肝硬化（PBC）在SS-AIHA患者中的患病率高于无SS-AIHA患者（p = 0.007）。伴有AIHA的pSS患者水肿、发热和肝脏受累的发生率显著高于无AIHA的pSS患者（分别为p = 0.035、p = 0.029、p = 0.024）。AIHA的pSS患者比没有AIHA的患者更容易发生白细胞减少症和血小板减少症（分别为p = 0.004和p = 0.001）。此外，SS-AIHA组的补体成分3（C3）和补体成分4（C4）水平显著较低（分别为p = 0.008和p = 0.037）。两者合计，我们的研究结果表明，pSS应考虑在AIHA的鉴别诊断，即使在没有干燥症状。在pSS患者中，PBC、血细胞减少或低补体血症的存在提示患AIHA的风险较高。
Autoimmune hemolytic anemia (AIHA) is an uncommon but clinically significant disorder in primary Sjogren'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.