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[未分化结缔组织病的临床特征:145例患者分析]

[Clinical features of undifferentiated connective tissue diseases: analysis of 145 patients].

作者信息

Zhang Xue-wu, Liu Xu, Li Zhan-guo

机构信息

Department of Rheumatology, People's Hospital of Beijing University, Beijing, China.

出版信息

Zhonghua Yi Xue Za Zhi. 2006 Sep 19;86(35):2458-61.

Abstract

OBJECTIVE

To investigate the clinical features and prognosis of undifferentiated connective tissue disease. (UCTD) METHODS: 1105 connective tissue disease (CTD) patients, including 751 cases of systemic lupus erythrematosus (SLE), 63 cases of systemic sclerosis (SSc), 103 cases of polymyositis/dermatomyositis (PM/DM), 159 cases of primary Sjögren's syndrome (pSS), and 29 cases of overlap syndrome (29), were randomly selected. The clinical data of these patients were analyzed to identify those who displayed the manifestations of UCTD as the onset manifestations so as to summarize the clinical manifestation, immunological parameters, and long term development of UCTD.

RESULTS

These 145 patients with UCTD developed SLE, SSc, SS, PM/DM, or overlap syndrome within two to five years. The patients with arthritis and arthralgia often developed into SLE. Raynaud's syndrome was often related to SSc. The patients with rash or face edema were more likely turned out to be PM/DM patients. The patients with dry eyes or dry mouth often developed into pSS patients.

CONCLUSION

UCTD can develop into various autoimmune diseases, such as SLE, SSc, pSS or PM/DM. Some clinical features of onset are related with the outcome.

摘要

目的

探讨未分化结缔组织病(UCTD)的临床特征及预后。方法:随机选取1105例结缔组织病(CTD)患者,其中包括751例系统性红斑狼疮(SLE)、63例系统性硬化症(SSc)、103例多发性肌炎/皮肌炎(PM/DM)、159例原发性干燥综合征(pSS)以及29例重叠综合征患者。分析这些患者的临床资料,以确定那些以UCTD表现为首发表现的患者,从而总结UCTD的临床表现、免疫参数及长期发展情况。

结果

这145例UCTD患者在两到五年内发展为SLE、SSc、SS、PM/DM或重叠综合征。有关节炎和关节痛的患者常发展为SLE。雷诺综合征常与SSc相关。有皮疹或面部水肿的患者更有可能成为PM/DM患者。有干眼或口干的患者常发展为pSS患者。

结论

UCTD可发展为多种自身免疫性疾病,如SLE、SSc、pSS或PM/DM。一些首发临床特征与结局相关。

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