Anna-Maria Hoffmann-Vold1, Ragnar Gunnarsson2, Torhild Garen2, Øyvind Midtvedt2, Øyvind Molberg2. 1. From the Department of Rheumatology, Oslo University Hospital-Rikshospitalet; and the Institute of Clinical Medicine, University of Oslo, Oslo, Norway.A-M. Hoffmann-Vold, Dr. med; R. Gunnarsson, Dr. med, Department of Rheumatology, Oslo University Hospital-Rikshospitalet and Institute of Clinical Medicine, University of Oslo; T. Garen; Ø. Midtvedt, MD, Department of Rheumatology, Oslo University Hospital-Rikshospitalet; Ø. Molberg, MD, Professor of Medicine, Department of Rheumatology, Oslo University Hospital-Rikshospitalet, and Institute of Clinical Medicine, University of Oslo. amhvold@ous-hf.no. 2. From the Department of Rheumatology, Oslo University Hospital-Rikshospitalet; and the Institute of Clinical Medicine, University of Oslo, Oslo, Norway.A-M. Hoffmann-Vold, Dr. med; R. Gunnarsson, Dr. med, Department of Rheumatology, Oslo University Hospital-Rikshospitalet and Institute of Clinical Medicine, University of Oslo; T. Garen; Ø. Midtvedt, MD, Department of Rheumatology, Oslo University Hospital-Rikshospitalet; Ø. Molberg, MD, Professor of Medicine, Department of Rheumatology, Oslo University Hospital-Rikshospitalet, and Institute of Clinical Medicine, University of Oslo.
Abstract
OBJECTIVE: To assess the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Classification Criteria for Systemic Sclerosis (SSc) on defined subgroups of SSc and in mixed connective tissue disease (MCTD) as an SSc-related disease. METHODS: The 2013 ACR/EULAR criteria were assessed in 425 consecutive patients suspected to have SSc and seen at Oslo University Hospital, and in the nationwide Norwegian MCTD cohort (n = 178). In the SSc group, 239/425 patients had disease duration < 3 years (in 82 of these, duration was < 1 yr). Patients were subgrouped as limited SSc (n = 294), diffuse SSc (n = 97), SSc sine scleroderma (n = 10), and early SSc (prescleroderma; n = 24). Item data were complete, except nailfold capillaroscopy and telangiectasia results, missing in the MCTD cohort. RESULTS: The 2013 ACR/EULAR SSc criteria were met by 409/425 patients (96%) in the SSc group. For comparison, only 75% (293/391) met the 1980 ACR SSc classification criteria. All the novel items in the 2013 ACR/EULAR criteria were frequent in the SSc cohort. Considering that there were missing data on 2 items, 10% (18/178) of the MCTD cohort met the 2013 ACR/EULAR criteria, giving an estimated specificity of 90% toward this SSc-like disorder. CONCLUSION: In our large and representative group of consecutive patients with SSc, the 2013 ACR/EULAR SSc criteria were more sensitive than the ACR 1980 criteria. However, the new criteria did not completely segregate SSc from MCTD, making specificity a potential issue.
OBJECTIVE: To assess the 2013 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) Classification Criteria for Systemic Sclerosis (SSc) on defined subgroups of SSc and in mixed connective tissue disease (MCTD) as an SSc-related disease. METHODS: The 2013 ACR/EULAR criteria were assessed in 425 consecutive patients suspected to have SSc and seen at Oslo University Hospital, and in the nationwide Norwegian MCTD cohort (n = 178). In the SSc group, 239/425 patients had disease duration < 3 years (in 82 of these, duration was < 1 yr). Patients were subgrouped as limited SSc (n = 294), diffuse SSc (n = 97), SSc sine scleroderma (n = 10), and early SSc (prescleroderma; n = 24). Item data were complete, except nailfold capillaroscopy and telangiectasia results, missing in the MCTD cohort. RESULTS: The 2013 ACR/EULAR SSc criteria were met by 409/425 patients (96%) in the SSc group. For comparison, only 75% (293/391) met the 1980 ACR SSc classification criteria. All the novel items in the 2013 ACR/EULAR criteria were frequent in the SSc cohort. Considering that there were missing data on 2 items, 10% (18/178) of the MCTD cohort met the 2013 ACR/EULAR criteria, giving an estimated specificity of 90% toward this SSc-like disorder. CONCLUSION: In our large and representative group of consecutive patients with SSc, the 2013 ACR/EULAR SSc criteria were more sensitive than the ACR 1980 criteria. However, the new criteria did not completely segregate SSc from MCTD, making specificity a potential issue.
Authors: J N Kimmel; D A Carlson; M Hinchcliff; M A Carns; K A Aren; J Lee; J E Pandolfino Journal: Neurogastroenterol Motil Date: 2016-02-27 Impact factor: 3.598
Authors: Caitrin M Coffey; Yasser A Radwan; Avneek S Sandhu; Cynthia S Crowson; Philippe R Bauer; Eric L Matteson; Ashima Makol Journal: J Scleroderma Relat Disord Date: 2021-06-23