Abstract:

Yang Ji, Cui Xiangxiang, Li Ming Department of Dermatology, Zhongshan Hospital, Fudan University, Shanghai 200032, China （Yang J, Li M）; Department of Dermatology, Chlidern′s Hospital of Shanghai, Shanghai 200040, China （Cui XX） Corresponding author: Li Ming, Email: li.ming@zs-hospital.sh.cn 【Abstract】 Objective To investigate clinical characteristics of systemic sclerosis （SSc）, and to explore the correlation between visceral involvement and autoantibodies. Methods Clinical data, laboratory examination and auxiliary examination findings were collected from 63 patients with SSc in the Department of Dermatology of Zhongshan Hospital affiliated to Fudan University between 2012 and 2015, and the correlation of autoantibodies with visceral involvement and clinical phenotypes was analyzed. Results The 63 patients with SSc included 29 with diffuse SSc, 29 with limited SSc and 5 with overlap syndrome. Of the 63 patients, 30 had pulmonary interstitial fibrosis, 32 had lung function impairment which manifested as reduced diffusing capacity for carbon monoxide, and 12 had increased pulmonary arterial pressure. Among 29 patients with anti- DNA topoisomeraseⅠ（SCL-70） antibody, 19 （65.52%） had pulmonary interstitial fibrosis, while 11 （32.35%） of 34 patients without anti-SCL-70 antibody had pulmonary interstitial fibrosis. Pulmonary interstitial fibrosis also occurred in 13 （68.42%） of 19 patients with anti-Sjogren′s syndrome antigen A （SSA） antibody, as well as in 17 （38.64%） of 44 patients without anti-SSA antibody. The incidence of pulmonary interstitial fibrosis was significantly higher in the patients with anti-SCL-70 or anti-SSA antibody than in the patients without anti-SCL-70 or anti-SSA antibody respectively （χ2 = 6.901, 4.720, respectively, both P < 0.05）. Among the 63 patients, 22 had fingertip ulcers, and 16 （72.73%） of the 22 patients had pulmonary interstitial fibrosis, while 14 （34.15%） of 41 patients without fingertip ulcers had pulmonary interstitial fibrosis, and the incidence of pulmonary interstitial fibrosis significantly differed between the two groups （χ2 = 8.544, P < 0.01）. Of the 63 patients, 50 （79.40%） initially presented with Raynaud′s phenomenon, and the average duration between the occurrence of Raynaud′s phenomenon and the diagnosis of SSc was 48.3 months. Conclusions Patients with SSc usually present with a high incidence of pulmonary interstitial fibrosis, which is complicated by lung function impairment and pulmonary hypertension. Raynaud′s phenomenon usually occurs as the initial symptom. There is a high incidence of pulmonary interstitial fibrosis in the patients with fingertip ulcers and anti-SCL-70 and anti -SSA antibodies.