title: Scleroderma renal crisis in a Swedish systemic sclerosis cohort: survival, renal outcome, and RNA polymerase III antibodies as a risk factor.
abstract: OBJECTIVE
  To study survival, renal outcome, and RNA polymerase III antibodies (RNAP Abs) as a risk factor for scleroderma renal crisis (SRC) in a Swedish cohort of systemic sclerosis (SSc) patients.
  
  
  METHODS
  SRC was diagnosed in 16 SSc patients during the period from 1982 to 2010. For comparison, 112 (seven for each SRC patient) SSc patients without SRC were included. RNAP Abs were detected by a fully automated fluoroenzyme immunoassay (EliA). Values greater than 15 μg/L were considered positive. Frozen serum samples from the time of diagnosis of SSc were used.
  
  
  RESULTS
  The 5- and 10-year survival rates were, respectively, 58% and 40% for SRC patients and 90% and 76% for patients without SRC (p < 0.001). The odds ratio (OR) for mortality was 4.39 [95% confidence interval (CI) 2.10-9.16, p < 0.001] in patients with SRC compared to those without SRC. Renal outcome was good in three patients. Haemodialysis was started in 10 patients and peritoneal dialysis in three. Renal function improved in three patients and dialysis was terminated. Four patients underwent renal transplantation. Seven SRC patients and nine without SRC were positive for RNAP Abs. Anti-RNAP Abs was a strong predictor of SRC. The sensitivity and specificity for development of SRC were 0.44 and 0.92, respectively. The OR for development of SRC was 8.90 (95% CI 2.68-29.6, p = 0.001) in RNAP-positive patients.
  
  
  CONCLUSIONS
  RNAP positivity is a strong risk factor for SRC. Renal outcome was variable and survival is still notably decreased.
authors: Department of Clinical Sciences, Section for Rheumatology, Lund University, Lund, Sweden. roger.hesselstrand@med.lu.se
authors: R
authors: R
authors: Hesselstrand
authors: A
authors: A
authors: Scheja
authors: D M
authors: DM
authors: Wuttge
mesh_terms: D000328:Adult; D000368:Aged; D000974:Antibodies, Antinuclear / Q000097:blood*; D016022:Case-Control Studies; D015331:Cohort Studies; D006801:Humans; D007668:Kidney / Q000503:physiopathology; D007674:Kidney Diseases / Q000209:etiology / Q000276:immunology / Q000401:mortality*; D008875:Middle Aged; D011379:Prognosis; D012320:RNA Polymerase III / Q000276:immunology*; D006435:Renal Dialysis; D012189:Retrospective Studies; D012307:Risk Factors; D012595:Scleroderma, Systemic / Q000276:immunology / Q000401:mortality*; D015996:Survival Rate; D013548:Sweden
chemical_list: D000974:Antibodies, Antinuclear; D012320:RNA Polymerase III