Neha Srivastava1, Marie Hudson2, Solène Tatibouet3, Mianbo Wang3, Murray Baron4, Marvin J Fritzler5. 1. Faculty of Medicine, McGill University, Jewish General Hospital, Montréal, Québec, Canada. 2. Faculty of Medicine, McGill University, Jewish General Hospital, Montréal, Québec, Canada; Division of Rheumatology, Jewish General Hospital, Montréal, Québec, Canada; Lady Davis Institute, Jewish General Hospital, Montréal, Québec, Canada. Electronic address: marie.hudson@mcgill.ca. 3. Lady Davis Institute, Jewish General Hospital, Montréal, Québec, Canada. 4. Faculty of Medicine, McGill University, Jewish General Hospital, Montréal, Québec, Canada; Division of Rheumatology, Jewish General Hospital, Montréal, Québec, Canada. 5. Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.
Abstract
OBJECTIVE: To describe the clinical characteristics and survival of anti-topoisomerase antibody positive (ATA+) limited cutaneous systemic sclerosis (lcSSc) and anti-centromere antibody positive (ACA+) diffuse cutaneous systemic sclerosis (dcSSc) patients in a large, multicenter SSc cohort. METHODS: Data from subjects in the Canadian Scleroderma Research Group (CSRG) cohort were extracted. Descriptive statistics were used to summarize the baseline characteristics, including sociodemographic, clinical and serological features of lcSSc and dcSSc, according to ATA+ and ACA+ subsets. Kaplan-Meier analysis was performed to investigate survival by subsets. RESULTS: Of the 551 subjects included in this study, 52 (9.4%) had ATA+ lcSSc and 91 (16.5%) had ACA+ dcSSc. Demographic and visceral organ involvement (e.g., gastrointestinal symptoms, interstitial lung disease, pulmonary hypertension, and scleroderma renal crisis) was associated with serologic status more so than with skin subset. On the other hand, calcinosis, joint and peripheral vascular manifestations were associated with skin rather than antibody status. Survival was associated with both skin and autoantibody subsets, with ATA + dcSSc associated with the worse survival compared to ATA+ lcSSc (p = 0.0115), ACA+ lcSSc (p = 0.0216) and ACA+ dcSSc (p = 0.0313). CONCLUSION: This study provides evidence that subsetting using antibody markers in addition to extent of skin involvement may predict clinical outcomes better than skin or serology alone in SSc. These findings can inform ongoing efforts to define more robust SSc subsets compared to those based on the extent of skin involvement or serology alone.
OBJECTIVE: To describe the clinical characteristics and survival of anti-topoisomerase antibody positive (ATA+) limited cutaneous systemic sclerosis (lcSSc) and anti-centromere antibody positive (ACA+) diffuse cutaneous systemic sclerosis (dcSSc) patients in a large, multicenter SSc cohort. METHODS: Data from subjects in the Canadian Scleroderma Research Group (CSRG) cohort were extracted. Descriptive statistics were used to summarize the baseline characteristics, including sociodemographic, clinical and serological features of lcSSc and dcSSc, according to ATA+ and ACA+ subsets. Kaplan-Meier analysis was performed to investigate survival by subsets. RESULTS: Of the 551 subjects included in this study, 52 (9.4%) had ATA+ lcSSc and 91 (16.5%) had ACA+ dcSSc. Demographic and visceral organ involvement (e.g., gastrointestinal symptoms, interstitial lung disease, pulmonary hypertension, and scleroderma renal crisis) was associated with serologic status more so than with skin subset. On the other hand, calcinosis, joint and peripheral vascular manifestations were associated with skin rather than antibody status. Survival was associated with both skin and autoantibody subsets, with ATA + dcSSc associated with the worse survival compared to ATA+ lcSSc (p = 0.0115), ACA+ lcSSc (p = 0.0216) and ACA+ dcSSc (p = 0.0313). CONCLUSION: This study provides evidence that subsetting using antibody markers in addition to extent of skin involvement may predict clinical outcomes better than skin or serology alone in SSc. These findings can inform ongoing efforts to define more robust SSc subsets compared to those based on the extent of skin involvement or serology alone.
Authors: Vincent Sobanski; Jonathan Giovannelli; Yannick Allanore; Gabriela Riemekasten; Paolo Airò; Serena Vettori; Franco Cozzi; Oliver Distler; Marco Matucci-Cerinic; Christopher Denton; David Launay; Eric Hachulla Journal: Arthritis Rheumatol Date: 2019-08-12 Impact factor: 10.995
Authors: Masataka Kuwana; Yannick Allanore; Christopher P Denton; Jörg H W Distler; Virginia Steen; Dinesh Khanna; Marco Matucci-Cerinic; Maureen D Mayes; Elizabeth R Volkmann; Corinna Miede; Martina Gahlemann; Manuel Quaresma; Margarida Alves; Oliver Distler Journal: Arthritis Rheumatol Date: 2022-02-13 Impact factor: 15.483