Ethnic Variations in Systemic Sclerosis Disease Manifestations, Internal Organ Involvement, and Mortality

Abstract

Objective.A multiethnic systemic sclerosis (SSc) cohort study to evaluate ethnic variations in disease manifestations, internal organ involvement, and survival.Methods.Adults who fulfilled the American College of Rheumatology/European League Against Rheumatism classification criteria for SSc between 1970 and 2017 were included. Self-reported ethnicity was categorized as European-descent white, Afro-Caribbean, Hispanic, Arab, East Asian, South Asian, First Nations, or Persian. The primary outcome was the time from diagnosis to death from all causes. Survival probabilities and median survival times were determined using Kaplan-Meier survival curves.Results.There were 1005 subjects evaluated, the majority of whom were European-descent white (n = 745, 74%), Afro-Caribbean (n = 58, 6%), South Asian (n = 70, 7%), and East Asian (n = 80, 8%). Compared to European-descent white subjects, East Asians less frequently had calcinosis (29% vs 9%, p = 0.002) and esophageal dysmotility (88% vs 69%, p = 0.002); Afro-Caribbeans more frequently had interstitial lung disease (31% vs 53%, p = 0.007); and First Nations subjects more frequently had diffuse cutaneous disease (35% vs 56%, p = 0.02) and diabetes (5% vs 33%, p = 0.03). We found no difference in the short-term survival across ethnicities. Hispanic subjects have better longterm survival (81.3%, 95% CI 63–100) compared to European-descent white subjects (55%, 95% CI 51–60). East Asians appear to have the longest median survival time (43.3 yrs) and Arabs the shortest median survival time (15 yrs). There was no significant difference in median survival times between Afro-Caribbean and European-descent white subjects (22.2 vs 22.6 yrs).Conclusion.Ethnic variations in some SSc disease manifestations are observed. However, this does not result in significant differences in short-term survival but may affect longterm survival.

Racial differences in systemic sclerosis disease presentation: a European Scleroderma Trials and Research group study

“…compared to our study [28]. The authors, however, did not find a difference in PH prevalence between patients of various ethnicities [28].…”

“…compared to our study [28]. The authors, however, did not find a difference in PH prevalence between patients of various ethnicities [28]. This goes in line with our finding that Asian patients treated outside Asia had a similar frequency of PH than the white centre-matched comparison group and hints towards a location-driven higher prevalence of PH in Asia.…”

“…An interesting result of our study is that Asian patients had an increased mortality in multivariable analysis compared to white patients. This finding however partly disappeared in the matched analyses again being in line with the Canadian study which also found no difference in the survival time between ethnicities including white and Asian patients [28]. However, the compared patients were all residing in Canada.…”

Predictors of progression in systemic sclerosis patients with interstitial lung disease

“…Despite the strong female predominance reported in SSc (female to male ratio 4.7:1), men with SSc are more likely to develop ILD than women with SSc (relative risk 1.24; 95% confidence interval 1.01-1.52) [31,32]. A large multi-ethnic observational study conducted in the setting of a universal healthcare system found that in comparison to white patients of European descent, patients of Afro-Caribbean descent with SSc more frequently have ILD (31% versus 53%; p=0.007) [33].…”

Esophageal Involvement and Determinants of Perception of Esophageal Symptoms Among South Koreans With Systemic Sclerosis

“…4 Specifically, East Asians had esophageal dysmotility (69.0%) less frequently compared with the white population (88.0%). 4 Esophageal manometry is the gold standard to evaluate esophageal dysmotility. The classic sclerosis esophagus is defined when SSc patients have no peristalsis in the distal esophagus and hypotensive lower esophageal sphincter (LES) pressure on manometric examination.…”