Liu Yuan, Chen Shiju, Sun Yuechi, Lin Qingyan, Liao Xining, Zhang Junhui, Luo Jiao, Qian Hongyan, Duan Lihua, Shi Guixiu
Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Xiamen University Medical College, Xiamen University, Xiamen, Fujian, China.
Medicine (Baltimore). 2016 Dec;95(50):e5565. doi: 10.1097/MD.0000000000005565.
To clarify clinical characteristics of immune thrombocytopenia (ITP) subsets associated with autoimmune diseases (AIDs).Five thousand five hundred twenty patients were reviewed retrospectively. One hundred four ITP patients were included for analysis. Clinical manifestations at first thrombocytopenic episode were recorded.Systemic lupus erythematosus (SLE) and primary Sjogren syndrome (pSS) accounted for a large part in AIDs associated with secondary ITP. SLE-ITP, pSS-ITP, and primary ITP (pITP) patients were different in several aspects in clinical and immunological characteristics. A subgroup of patients in pITP patients with some obvious autoimmune features (defined as AIF-ITP) such as positive ANA but failing to meet the diagnosis criteria now used for a specific kind of connective tissue diseases were also different with other pITP patients in some immunological features, indicating the difference in the pathogenesis mechanism of those autoimmune featured ITP patients.ITP patients were heterogeneous in clinical characteristics. Further study about the different pathogenesis of ITP subsets especially those AIF-ITP patients who only presented with thrombocytopenia will help us have a better understanding of pathogenesis of ITP and a better management of ITP patients.
为明确与自身免疫性疾病(AIDs)相关的免疫性血小板减少症(ITP)亚组的临床特征。对5520例患者进行回顾性研究。纳入104例ITP患者进行分析。记录首次血小板减少发作时的临床表现。系统性红斑狼疮(SLE)和原发性干燥综合征(pSS)在与继发性ITP相关的AIDs中占很大比例。SLE-ITP、pSS-ITP和原发性ITP(pITP)患者在临床和免疫特征的几个方面存在差异。pITP患者中具有一些明显自身免疫特征(定义为AIF-ITP)的亚组,如ANA阳性但不符合目前用于特定结缔组织病的诊断标准者,在一些免疫特征方面也与其他pITP患者不同,这表明这些具有自身免疫特征的ITP患者发病机制存在差异。ITP患者在临床特征上具有异质性。进一步研究ITP亚组尤其是那些仅表现为血小板减少的AIF-ITP患者的不同发病机制,将有助于我们更好地理解ITP的发病机制并更好地管理ITP患者。