What have multicentre registries across the world taught us about the disease features of systemic sclerosis?

Abstract

The aim of this study is to compare the clinical features, mortality and causes of death of systemic sclerosis (SSc) patients in four large multicentre registries. Methods: Patients seen at least once in the Australian Scleroderma Cohort Study (ASCS) (n = 1714), the Canadian Scleroderma Research Group (CSRG) (n = 1628), the European League Against Rheumatism Scleroderma Trials and Research (EUSTAR) Network (n = 13,996) and the Systemic Sclerosis Cohort in Singapore (SCORE) (n = 500) before August 2016 were included. Clinical manifestations and survival in cohorts and disease subtypes were compared. Results: Among 17,838 SSc patients, most were female (86.1%), Caucasian (84.6%) and had the limited cutaneous subtype (lcSSc) (65.0%). The anti-centromere autoantibody was the most prevalent (37.6%). More patients in SCORE had the diffuse subtype (dcSSc) (49.3%) and Scl-70 autoantibody (38.8%) (p<0.001). Patients with dcSSc were more likely to be younger and male (p<0.001) and have shorter disease duration, more calcinosis, tendon friction rubs and synovitis (all p<0.001). Interstitial lung disease (ILD) occurred more frequently in dcSSc but prevalence of pulmonary arterial hypertension (PAH) was similar in both subtypes. More deaths occurred among SCORE patients who had the shortest median survival (p<0.001). The survival of patients with early disease, males and those with dcSSc was shorter than that of patients with prevalent disease, female gender and lcSSc, respectively. SSc-related complications accounted for more than 50% of deaths, with PAH and ILD being the most common. Conclusions: This meta-cohort of SSc patients, the largest reported to date, provides insights into the impact of race and sex on disease manifestations and survival and confirms the early mortality in this disease.

Development and validation of a patient-reported outcome measure for systemic sclerosis: the EULAR Systemic Sclerosis Impact of Disease (ScleroID) questionnaire

“…SSc has one of the highest morbidities and case-specific mortalities amongst the connective tissue diseases [2,3]. Overall, general health (as measured by the SF-36 and EQ-5D questionnaires), as well as quality of life and functional abilities (as measured by the Health Assessment Questionnaire Disability Index, HAQ-DI) are significantly reduced in SSc [4][5][6].…”

Evaluation of Scleroderma Clinical Trials Consortium training recommendations on modified Rodnan skin score assessment in scleroderma

“…Further, the findings from our study may be generalized to patients of Asian descent. Previous studies demonstrated no significant differences in initial or peak mRSS between SSc patients of Asian descent and their European counterparts …”

Clinical and laboratory profile of juvenile-onset systemic sclerosis in a Brazilian cohort

“…SSc is more frequent in the female gender and in the Caucasian race, and usually starts at an adult age. 3 Its prevalence seems to be higher in North America and Australia compared to Europe and Japan. 4,5 It is important to consider that juvenile-onset SSc, despite being a rare disease, can present a distinct clinical and immunological profile, with a distinct outcome, when compared with adult-onset SSc patients.…”

“…SSc is more frequent in the female gender and in the Caucasian race, and usually starts at an adult age. 3 Its prevalence seems to be higher in North America and Australia compared to Europe and Japan. 4,5…”