无硬皮的系统性硬化病一例并文献复习

A case of systemic sclerosis sine scleroderma and a literature review

目的无硬皮的系统性硬化病（ssSSc）是SSc中一个罕见类型，临床极为少见，比较了解ssSSc与其他类型SSc异同。方法报道1例ssSSc患者，表现有雷诺现象、食管、肠道、肺脏受累，且ANA阳性，抗拓扑异构酶70（Scl-70）抗体阳性，但无任何皮肤硬化表现，同时查阅复习文献了解ssSSc特点。结果ssSSc与局限性皮肤型系统性硬化病（1cSSc）内脏受累无差别，肺动脉高压的患病率有增高趋势，钙质沉着、指端溃疡、肢端溶解发生率较低，ssSSc和lcSSc间质性肺病的发生率均比弥漫性皮肤型系统性硬化病（dcSSc）低。结论ssSSc患者虽无皮肤硬化，但内脏受累亦较为广泛，早期诊断、早期治疗极为重要。

Objective Systemic sclerosis sine scleroderma （ssSSc） is a rare type of systemic sclerosis, comparison between ssSSc and other types of systemic sclerosis （SSc）, ssSSc is carried out in this study. Methods We reported a patient with Raynaud＇s phenomenon, esophagus, intestine and lung involvement, positive ANA, anti- Scl-70 antibody, but had no skin sclerosis. Results For visceral involvement, ssSSc was not different from limited cutaneous systemic sclerosis （lcSSc）, The incidence of pulmonary hypertension was higher in patients with ssSSc, lower with calcinosis, finger ulcer and acral dissolution, and the incidence of ssSSc and lcSSc interstitial lung disease was higher than that of diffuse cutaneous systemic sclerosis, （dcSSc）. Conclusion Ahhough ssSSc do not have skin sclerosis, however, visceral involvement is more extensive. Early diagnosis and treatment is extremely important.