Haifa Al-Sheikh1,2, Zareen Ahmad1,2, Sindhu R Johnson3,4. 1. From the Toronto Scleroderma Program, Sinai Health Systems, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada; Division of Rheumatology, Department of Medicine, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia. 2. H. Al-Sheikh, MD, Toronto Scleroderma Program, Sinai Health Systems, Division of Rheumatology, Department of Medicine, University of Toronto, and Division of Rheumatology, Department of Medicine, King Faisal Specialist Hospital & Research Center; Z. Ahmad, MD, Toronto Scleroderma Program, Sinai Health Systems, Division of Rheumatology, Department of Medicine, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Sinai Health Systems, Toronto Western Hospital, Division of Rheumatology, Department of Medicine; Institute of Health Policy, Management and Evaluation, University of Toronto. 3. From the Toronto Scleroderma Program, Sinai Health Systems, Toronto Western Hospital, Division of Rheumatology, Department of Medicine, University of Toronto; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Ontario, Canada; Division of Rheumatology, Department of Medicine, King Faisal Specialist Hospital & Research Center, Riyadh, Saudi Arabia. Sindhu.Johnson@uhn.ca. 4. H. Al-Sheikh, MD, Toronto Scleroderma Program, Sinai Health Systems, Division of Rheumatology, Department of Medicine, University of Toronto, and Division of Rheumatology, Department of Medicine, King Faisal Specialist Hospital & Research Center; Z. Ahmad, MD, Toronto Scleroderma Program, Sinai Health Systems, Division of Rheumatology, Department of Medicine, University of Toronto; S.R. Johnson, MD, PhD, Toronto Scleroderma Program, Sinai Health Systems, Toronto Western Hospital, Division of Rheumatology, Department of Medicine; Institute of Health Policy, Management and Evaluation, University of Toronto. Sindhu.Johnson@uhn.ca.
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.
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.
Authors: Oliver Distler; Shervin Assassi; Vincent Cottin; Maurizio Cutolo; Sonye K Danoff; Christopher P Denton; Jörg H W Distler; Anna-Maria Hoffmann-Vold; Sindhu R Johnson; Ulf Müller Ladner; Vanessa Smith; Elizabeth R Volkmann; Toby M Maher Journal: Eur Respir J Date: 2020-05-14 Impact factor: 16.671