title: Performance of the 2013 American College of Rheumatology/European League Against Rheumatism Classification Criteria for Systemic Sclerosis (SSc) in large, well-defined cohorts of SSc and mixed connective tissue disease.
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.
  
  
  CONCLUSIONS
  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.affiliation: 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.
authors.forename: Anna-Maria
authors.initials: AM
authors.lastname: Hoffmann-Vold
authors.affiliation: 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.
authors.forename: Ragnar
authors.initials: R
authors.lastname: Gunnarsson
authors.affiliation: 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.
authors.forename: Torhild
authors.initials: T
authors.lastname: Garen
authors.affiliation: 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.
authors.forename: Øyvind
authors.initials: Ø
authors.lastname: Midtvedt
authors.affiliation: 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.
authors.forename: Øyvind
authors.initials: Ø
authors.lastname: Molberg
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D005260:Female; D006801:Humans; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D008947:Mixed Connective Tissue Disease / Q000145:classification / Q000175:diagnosis*; D012219:Rheumatology; D012595:Scleroderma, Systemic / Q000145:classification / Q000175:diagnosis*; D012680:Sensitivity and Specificity; D055815:Young Adult
keywords: AUTOIMMUNE DISEASES; CONNECTIVE TISSUE DISEASES; EPIDEMIOLOGY; SYSTEMIC SCLEROSIS