Survival rate, causes of death, and risk factors in systemic sclerosis: a large cohort study

Abstract

To investigate the clinical pattern, survival rate, causes of death and risk factors in a large cohort of Chinese Han patients with systemic sclerosis (SSc). Inpatients treated from 2002 to 2014 were included in this study. Patients were classified into diffuse cutaneous SSc (dcSSc), limited cutaneous SSc (lcSSc), and SSc-overlap syndrome groups. Data were analyzed using Chi-squared tests, Kaplan-Meier curves, log-rank tests, and Cox proportional hazards modeling. Among a total of 201 patients, dcSSc (50.2%) was the major subtype, followed by lcSSc (30.3%) and SSc-overlap (19.4%). Interstitial lung disease (ILD, 148/201, 74%) was the most frequent organ involvement. The overall survival rates were 98% and 95% at 5 and 10 years, respectively. The overall standard mortality ratio (SMR) was 2.22. The most common cause of death was ILD combined with infection (8/16, 50%), followed by kidney failure (2/16, 12.5%). On crude analysis, pulmonary hypertension, ILD, cardiac involvements, renal involvements, and digital ischemia were associated with poor prognosis. On multivariate analysis, pericardial effusion (p = 0.000) and digital ischemia (p = 0.016) were independent prognostic factors of death. The mortality rate of patients with SSc is mildly increased in comparison with the general population. ILD is the most common systemic involvement and the principal cause of death in SSc. Pericardial effusion and digital ischemia are independent factors associated with death.

Sine Scleroderma, Limited Cutaneous and Diffused Cutaneous Systemic Sclerosis Survival and Predictors of Mortality

“…Our overall survival rates at 1 year, 3 years, 5 years, 10 years were consistent with those reported previously [8,9,11,15,18]. In addition, we have shown a non-significant higher mortality rate from the diffuse cutaneous sub-type vs. the limited cutaneous sub type and the sine scleroderma sub type of SSc, in line with the published literature [8,11,12,16,17,[50][51][52].…”

“…However, 46.4% of the deaths in our cohort were directly attributable to SSc. Our results were consistent with the data from the EUSTAR cohort, finding that nowadays the two main causes of SSc-related were ILD and PH [3,6,12,15,17,50,51,60]. SSc GI-related mortality were frequent in our cohort (18.2%) and this cause of death represented 7.6% of deaths in metanalysis of Rubi-Rivas et al [8].…”

“…In our study, cardiac involvement was a composite outcome including LVEF <50%, and/or a TTE anomaly (pericarditis, valvulopathy or diastolic dysfunction), and/or an ECG anomaly (arrhythmia or conduction blocks), which was strongly associated with a 2.9-fold increase mortality risk (HR=2.86 95% CI 1.54-5.41). Finally, we confirmed that systemic inflammation was a major pejorative prognostic factor of mortality (HR: 2.13 95%CI 1.11-5.41) like reported by few studies [11,15,51].…”

Prevalence and risk factors of systemic sclerosis‐associated interstitial lung disease in East Asia: A systematic review and meta‐analysis

“…The pooled SSc‐ILD prevalence was 56% (95% CI 49‐63) (Figure 2), which was stable in the sensitivity analysis (Figure 3). 11‐37 Heterogeneity across studies was high ( I 2 = 96.1%). There were significant differences in the prevalence of SSc‐ILD for subgroups stratified by region, study quality, SSc classification criteria, and publication year (Table 2).…”

Renal Involvement in Systemic Sclerosis: An Update

“…Cannon et al [7] found that up to 50% of asymptomatic patients have clinical markers indicative of renal dysfunction, such as proteinuria, an increased creatinine concentration, or hypertension. Various studies suggest a strong association between renal involvement and worse patient outcomes in SSc [3, 8]. In addition, a multinational SSc inception cohort study found renal crisis to be one of the predictors of early mortality in SSc patients [9].…”