title: Survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis.
abstract: OBJECTIVE
  To determine survival and causes of death in an unselected and complete cohort of Norwegian patients with systemic sclerosis (SSc) compared to the background population.
  
  
  METHODS
  Multiple methods were used to identify every patient with SSc living in southeast Norway, with a denominator population of 2,707,012, between 1999 and 2009. All patients who met either the American College of Rheumatology criteria or the Medsger and LeRoy criteria for SSc were included. Every patient was matched for sex and age with 15 healthy controls drawn from the national population registry. Vital status at January 1, 2010, was provided for patients and controls by the national population registry. Causes of death were obtained from death certificates and by chart review.
  
  
  RESULTS
  Forty-three (14%) of 312 patients with SSc died during the study period. The standardized mortality rate (SMR) was estimated to be 2.03 for the entire cohort and 5.33 for the subgroup with diffuse cutaneous (dc) SSc. The 5- and 10-year survival rates were 91% and 70%, respectively, for dcSSc and 98% and 93% for limited cutaneous (lc) SSc. Causes of death were related to SSc in 24/43 (56%) patients, mostly cardiopulmonary diseases (n = 13), including pulmonary hypertension (n = 8). Factors associated with fatal outcome included male sex, dcSSc, pulmonary hypertension, and interstitial lung disease.
  
  
  CONCLUSIONS
  Compared to the Norwegian background population, our cohort of 312 unselected patients with SSc had decreased survival. The survival rates observed were, however, better than those previously reported from SSc referral centers.
authors: Department of Rheumatology, Oslo University Hospital - Rikshospitalet, Norway. amhvold@ous-hf.no
authors: Anna-Maria
authors: AM
authors: Hoffmann-Vold
authors: Øyvind
authors: Ø
authors: Molberg
authors: Øyvind
authors: Ø
authors: Midtvedt
authors: Torhild
authors: T
authors: Garen
authors: Jan Tore
authors: JT
authors: Gran
mesh_terms: D000328:Adult; D000368:Aged; D002423:Cause of Death; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000150:complications / Q000401:mortality*; D017563:Lung Diseases, Interstitial / Q000150:complications / Q000401:mortality*; D008297:Male; D008875:Middle Aged; D009664:Norway / Q000453:epidemiology; D012042:Registries; D012595:Scleroderma, Systemic / Q000150:complications / Q000401:mortality*; D012737:Sex Factors; D015996:Survival Rate
keywords: CONNECTIVE TISSUE DISEASES; EPIDEMIOLOGY; MORTALITY; SURVIVAL; SYSTEMIC SCLEROSIS