摘要
目的:了解混合性结缔组织病(mixed connective tissue disease,MCTD)的临床特点及其转归。方法:回顾性分析1990年至2008年在北京大学人民医院风湿免疫科收治的91例MCTD患者,分析其临床表现、实验室检查及临床辅助检查特点,并对其进行随访,依据其不同转归结果进行分组比较。结果:MCTD患者首诊时雷诺现象、关节痛、关节炎、发热、乏力、抗核抗体(antinuclear antibodies,ANA)阳性和抗核糖核蛋白抗体(ribosenuclear protein an-tibodies,RNP)阳性的发生率高。91例患者中有63例完成随访,死亡6例,失访22例。63例MCTD患者经过1～6年随访,8例明确诊断为系统性红斑狼疮(systemiclupuserythematosus,SLE),2例为抗中性粒细胞胞浆抗体(anti-neu-trophil cytoplasmic antibodyes,ANCA)相关性小血管炎(ANCA-associated vasculitis,AAV),2例为类风湿关节炎(rheumatoid arthritis,RA),1例为原发性干燥综合征(primary Sjgren’s syndrome,pSS),50例仍为MCTD。发展为SLE的患者发病初期脱发,蛋白尿,血小板下降,低补体血症的发生率较高。结论:MCTD可发展为明确的自身免疫性疾病,其转归与发病时的临床特点有关。
Objective：To investigate the clinical features and prognosis of mixed connective tissue disease（MCTD）.Methods： Clinical,laboratory and instrumental examination information of 91 patients with MCTD,who were diagnosed between 1990 to 2008 in Peking University People＇s Hospital,were collected and analyzed retrospectively.These patients were following-up,and different outcoms compared.Results： The most common manifestations of MCTD patients were Raynaud phenomenon,arthralgia,arthritis,fever,acratia,positivities of antinuclear antibodies（anti-ANA） and ribosenuclear protein antibodies（anti-RNP）,which were 94.5%,78%,46.2%,48.4%,53.9%,100% and 100%,respectively.Six patients died,and 22 patients were lost in the follow-up after discharge.Among the remaining 63 patients,8 developed into systemic lupus erythomatosus（SLE）,and 2 into antineutrophil cytoplasmic antibodies-associated vasculitis（AAV）,1 into primary Sjgren＇s syndrome（pSS）,and 2 into rheumatoid arthritis（RA） at one to six years after diagnosis of MCTD.The patients who initially manifested as alopecia,proteinuria,thrombocytopenia,low complement were more likely to develop into SLE.Conclusion：MCTD can develop into various autoimmune diseases,such as SLE,pSS,RA,AAV.Some clinical features can probably predict future outcomes.
出处
《北京大学学报（医学版）》
CAS
CSCD
北大核心
2012年第2期270-274,共5页
Journal of Peking University:Health Sciences
关键词
混合性结缔组织病
病理状态
体征和症状
治疗结果
随访研究
Mixed connective tissue disease
Pathological conditions
signs and symptoms
Tueatment outcome
Follow-up studies
