title: Systemic sclerosis sine scleroderma: a multicenter study of 1417 subjects.
abstract: OBJECTIVE
  To describe the clinical and serological features of systemic sclerosis sine scleroderma (ssSSc) in a multicentered SSc cohort.
  
  
  METHODS
  Data from 1417 subjects in the Canadian Scleroderma Research Group registry were extracted to identify subjects with ssSSc, defined as SSc diagnosed by an expert rheumatologist, but without any sclerodactyly or skin involvement prior to baseline study visit or during followup. Clinical and serological features of ssSSc subjects were compared to limited (lcSSc) and diffuse cutaneous SSc (dcSSc) subjects.
  
  
  RESULTS
  At the first registry visit, only 57 subjects (4.0%) were identified as having ssSSc. Of these, 30 (2.1%) were reclassified as lcSSc within 1.9 years. Thus, only 27 ssSSc subjects (1.9%) remained, with mean followup of 2.4 years. Clinical profiles of ssSSc were generally similar or milder compared to lcSSc, and milder than dcSSc, including rates of interstitial lung disease (25.9% ssSSc, 25.4% lcSSc, 40.3% dcSSc). Patients with ssSSc had serological profiles similar to those with lcSSc, including high rates of anticentromere antibodies (50.0% ssSSc, 47.5% lcSSc, 12.1% dcSSc), and low rates of antitopoisomerase I (16.7% ssSSc, 7.0% lcSSc, 21.8% dcSSc) and anti-RNA polymerase III (0 ssSSc, 11.1% lcSSc, 34.9% dcSSc).
  
  
  CONCLUSIONS
  The condition ssSSc is rare and resembles lcSSc. These observations suggest that ssSSc is most likely a forme fruste of lcSSc, and that the absence of skin involvement may in part be related to misclassification arising from early or subtle skin involvement. There is little evidence to consider ssSSc as a distinct clinical or serological subset of SSc.
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital.
authors.forename: Sehriban
authors.initials: S
authors.lastname: Diab
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital.
authors.forename: Nathaniel
authors.initials: N
authors.lastname: Dostrovsky
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital. marie.hudson@mcgill.ca.
authors.forename: Marie
authors.initials: M
authors.lastname: Hudson
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital.
authors.forename: Solène
authors.initials: S
authors.lastname: Tatibouet
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital.
authors.forename: Marvin J
authors.initials: MJ
authors.lastname: Fritzler
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital.
authors.forename: Murray
authors.initials: M
authors.lastname: Baron
authors.affiliation: From the Department of Medicine, McMaster University, Hamilton, Ontario; Department of Medicine, McGill University; Division of Rheumatology, Lady Davis Institute, Jewish General Hospital, Montréal, Quebec; Faculty of Medicine, University of Calgary, Calgary, Alberta, Canada.S. Diab, MD; N. Dostrovsky, MD; N. Khalidi, MD, Department of Medicine, McMaster University; M. Hudson, MD, MPH; Department of Medicine, McGill University, Division of Rheumatology, Lady Davis Institute, Jewish General Hospital; S. Tatibouet, MSc, Lady Davis Institute, Jewish General Hospital; M.J. Fritzler, PhD, MD, Faculty of Medicine, University of Calgary; M. Baron, MD, Department of Medicine, McGill University, Division of Rheumatology, Jewish General Hospital.
authors.forename: Nader
authors.initials: N
authors.lastname: Khalidi
mesh_terms: D000328:Adult; D017677:Age Distribution; D002170:Canada / Q000453:epidemiology; D015331:Cohort Studies; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D015994:Incidence; D008297:Male; D008875:Middle Aged; D012042:Registries; D018570:Risk Assessment; D045743:Scleroderma, Diffuse / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D045745:Scleroderma, Limited / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy; D012595:Scleroderma, Systemic / Q000175:diagnosis* / Q000453:epidemiology* / Q000628:therapy; D012698:Serologic Tests / Q000379:methods; D012720:Severity of Illness Index; D017678:Sex Distribution; D016019:Survival Analysis
keywords: DIFFUSE SYSTEMIC SCLEROSIS; LIMITED SYSTEMIC SCLEROSIS; SCLERODERMA SINE SCLERODERMA; SYSTEMIC SCLEROSIS