系统性硬化症25例临床和免疫学分析

目的 分析系统性硬化症（SSc）的临床表现，探讨其免疫学改变的意义。方法 对25例SSc患者的临床和实验室资料进行回顾性分析。结果 25例患者均有不同程度的皮肤受累，有雷诺现象18例（72％），内脏受累按比例高低依次为肺（52％）、心脏（48％）、食管（24％），合并其他自身免疫性疾病4例，死亡3例，其中死于心律失常2例；ANA阳性19例（76％），抗ds-DNA抗体阳性4例（16％），抗Scl-70抗体阳性3例（12％）。结论 SSc常累及多个系统器官，并存在多种免疫学异常，ANA阳性与SSc密切相关，心脏损害是本症常见的死亡原因。

Clinical and immunological features of 25 patients with systemic sclerosis

Objective To study clinical manifestation and immunological abnormality of the patients who suffered from systemic sclerosis(SSc).Methods The clinical and laboratory data of 25 patients with SSc were retrospectively analyzed.Results All patients manifested skin damages in different degrees, Raynaud's phenomenon was found in 18 patients (72%) ,the rate of involvement of the lung, heart and esophagus were 52%,48% and 24%. Four patients had other autoimmunity pictures. Two of 3 dead patients died of cardiac arrhythmia. The positive rates of antinuclear antibody (ANA), anti ds DNA and anti Scl 70 were 76%(19/25),16%(4/25) and 12%(3/25),respectively. Conclusion The patients of SSc present several systemic involvement and abnormal immune reaction. ANA positive is closely related with SSc. Cardiac damage is common lethal cause of SSc.