A case of systemic sclerosis sine scleroderma and a literature review

VernacularTitle:无硬皮的系统性硬化病一例并文献复习
Author: Guizhi ZHANG ¹ ; Ning TIE ; Hongbin LI
Author Information

1. 内蒙古医科大学附属医院风湿免疫科
Keywords: Scleroderma,systemic; Systemic sclerosis sine scleroderma; Scleroderma,localized
From: Chinese Journal of Rheumatology 2017;21(12):833-836
CountryChina
Language:Chinese
Abstract: 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 Raynand'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 Although ssSSc do not have skin sclerosis,however,visceral involvement is more extensive.Early diagnosis and treatmeat is extremely important.