Clinical Characteristics And Prognostic Risk Factors Of Patients With Systemic Sclerosis

Objective The study was aimed to investigate the clinical characteristics and survival of SSc patients to provide a clinical basis for understanding SSc.Methods The research was retrospectively collected data of SSc patients who were admitted to our hospital from October 1,2012 to January 1,2019.Demographics,clinical,laboratory and risk factors associated with mortality were analyzed.According to the skin involvement,patients were divided into Ic SSc and dc SSc.Patients were followed up and then divided into the survivor-grous and the deceased according to the outcome of the follow-up.Kaplan-meier method was used to estimate the 1,3,5 and10 years survival rates.Cox proportional-hazards regression model was used to identify independent risk factors for mortality.Results1.A total of 723 SSc patients were identified,the mean age at diagnosis was 51.85±12.88 years and the median disease duration was 8 months.There were more patients from those cities,including Nanning,Hechi,Laibin and Chongzuo.In this study,49.65% zhuang chinese SSc patients were included and han chinese patients was 46.61%.2.Dc SSc was more common in patients hospitalized.The prevalence of patients with lung interstitial disease(ILD),pulmonary hypertension(PAH),renal crisis were 62.4%、18.7%、3.6%,respectively.Compared with Ic SSc,dc SSc patients were more common in males,and higher incidence of shortness of breath after activity,chest tightness,digital ulcers,limited activity of joint,PAH,renal crisis and restrictive ventilation dysfunction,anti-scl-70 antibody positive,and anti-nucleolar ANA type(P<0.05).3.The overall SSc mortality rate was 15.7%.Compared with the survivor,non-survivor of SSc patients were older,more chest tightness,shortness of breath after activity,ILD,PAH,renal crisis,and restricted ventilation dysfunction.(P<0.05).4.The overall survival rates of SSc patients were 94.3%,88.2%,84.9% and 77.6% at 1,3,5 and 10 years respectively;there was no significant difference in survival rate between dc SSc and Ic SSc.Cox proportional-hazards regression analysis showed that age ≥50 years old,symptoms of shortness of breath after activity and chest tightness,with ILD,PAH,renal crisis and restrictive ventilation dysfunction were related to mortality in SSc patients,and results of multivariate analysis showed that renal crisis,PAH and restrictive ventilation dysfunction were independent risk factors for death.Conclusion1.The majority of SSc patients are female,most of them are farmers,and the number of patients of Zhuang and Han Chinese similar,which is related to the geographical location.Dc SSc is more common in the hospitalized SSc patients,the prevalence of ILD was 62.4% and PAH was18.7%.2.5 and 10 years survival rates of SSc patients were 84.9% and 77.6%,respectively.Age ≥50 years,shortness of breath after activity and chest tightness,ILD,PAH,renal crisis,and restrictive ventilation dysfunction were related to mortality in SSc patients.And renal crisis,PAH,and restrictive ventilation dysfunction were independent risk factors for death.