title: Systemic sclerosis sine scleroderma: distinct features in a large Brazilian cohort.
abstract: OBJECTIVE
  Systemic sclerosis sine scleroderma (ssSSc) is an infrequent SSc variant characterized by visceral and immunological manifestations of SSc in the absence of clinically detectable skin involvement. We sought to delineate the characteristics of ssSSc in a cohort of Brazilian patients and contrast them with those in the literature.
  
  
  METHODS
  SSc patients seen at two academic medical centres in Brazil were retrospectively analysed. Patients were classified as ssSSc if they presented with RP, positive ANAs and at least one visceral involvement typical of SSc in the absence of skin thickening. Demographics, clinical and laboratory data were obtained by chart review. Literature review was performed by searching available original studies up until June 2012.
  
  
  RESULTS
  Among the 947 consecutive patients with SSc, 79 (8.3%) were classified as ssSSc. Oesophagus was the most frequently affected organ (83.1%), followed by pulmonary involvement (63.2%). Compared with the limited cutaneous form of SSc, telangiectasia was the only variable significantly different after multivariate logistic regression analyses (odds ratio 0.46; 95% CI 0.27, 0.81). Compared with the diffuse cutaneous form of SSc, multivariate analyses revealed that ssSSc patients were less likely to be male (odds ratio 0.15; 95% CI 0.04, 0.57), have digital ulcers (odds ratio 0.26; 95% CI 0.13, 0.51) or anti-Scl70 antibodies (odds ratio 0.19; 95% CI 0.07, 0.55) and less frequently treated with CYC (odds ratio 0.23; 95% CI 0.12, 0.43). These features were comparable to those in the published literature.
  
  
  CONCLUSIONS
  In this series, patients with ssSSc had a relatively mild disease with good prognosis.
authors: Division of Rheumatology, University of São Paulo School of Medicine, São Paulo, Brazil.
authors: Roberta Gonçalves
authors: RG
authors: Marangoni
authors: Luiza Fuoco
authors: LF
authors: Rocha
authors: Ana Paula T
authors: AP
authors: Del Rio
authors: Natalino H
authors: NH
authors: Yoshinari
authors: João Francisco
authors: JF
authors: Marques-Neto
authors: Percival D
authors: PD
authors: Sampaio-Barros
mesh_terms: D000046:Academic Medical Centers; D000328:Adult; D017677:Age Distribution; D001938:Brazil / Q000453:epidemiology; D015331:Cohort Studies; D005260:Female; D006801:Humans; D015994:Incidence; D016015:Logistic Models; D008297:Male; D008875:Middle Aged; D015999:Multivariate Analysis; D011379:Prognosis; D035583:Rare Diseases; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000145:classification / Q000453:epidemiology* / Q000473:pathology*; D012720:Severity of Illness Index; D017678:Sex Distribution
keywords: epidemiology; systemic sclerosis sine scleroderma