摘要
目的了解发展为明确自身免疫性疾病的未分化结缔组织病(UCTD)的临床特点。方法随机选取1105份住院病例,其中系统性红斑狼疮(SLE)751例、系统性硬化症(SSc)63例、多发性肌炎/皮肌炎(PM/DM)103例,干燥综合征(pSS)159例,重叠综合征29例。对发病时符合UCTD 患者的临床特点及免疫学指标进行分析。结果 75例 SLE、27例 SSc、15例 PM/DM、26例 pSS和2例重叠综合征患者,共176例是由 UCTD 经过平均2～5年发展而来。发展为 SLE 的患者在发病时以关节痛或关节炎最为常见,分别为56%(42/75)和28%(21/75),抗核抗体阳性率为58.7%(44/75);发展为 SSc 的患者中,85.2%(23/27)以雷诺现象为首发症状;发展为 PM/DM 的患者以皮疹或颜面浮肿为主要表现分别占40%(6/15)和33.3%(5/15);而发展为 pSS 的患者则以口干或眼干为常见的表现,占53.8%(14/26)。结论 UCTD 可发展为明确的自身免疫性疾病/弥漫性结缔组织病,包括 SLE、SSc、pSS、PM/DM 及重叠综合征。UCTD 的转归与发病时的临床特点有关。
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 erythromatosus （SLE）, 63 cases of systemic sclerosis （SSc）, 103 cases of polymyositis/dermatomyositis （PM/DM）, 159 cases of primary Sjoegren＇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.
出处
《中华医学杂志》
CAS
CSCD
北大核心
2006年第35期2458-2461,共4页
National Medical Journal of China
关键词
未分化结缔组织病
临床分析
Undifferentiated connective tissue diseases
Clinical analysis
