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Scleroderma renal crisis in a Swedish systemic sclerosis cohort: survival, renal outcome, and RNA polymerase III antibodies as a risk factor.

Hesselstrand, Roger LU ; Scheja, Agneta LU and Wuttge, Dirk LU (2012) In Scandinavian Journal of Rheumatology 41. p.39-43
Abstract
Objectives: 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 fluoroenzymeimmunoassay (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... (More)
Objectives: 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 fluoroenzymeimmunoassay (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. (Less)
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author
organization
publishing date
type
Contribution to journal
publication status
published
subject
in
Scandinavian Journal of Rheumatology
volume
41
pages
39 - 43
publisher
Taylor & Francis
external identifiers
  • wos:000299696100007
  • pmid:22044051
  • scopus:84856466047
ISSN
1502-7732
DOI
10.3109/03009742.2011.610032
language
English
LU publication?
yes
id
4039c615-cb11-47c6-9f31-1187c68fe710 (old id 2221334)
alternative location
http://www.ncbi.nlm.nih.gov/pubmed/22044051?dopt=Abstract
date added to LUP
2011-12-02 18:17:00
date last changed
2017-10-22 04:56:00
@article{4039c615-cb11-47c6-9f31-1187c68fe710,
  abstract     = {Objectives: 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 fluoroenzymeimmunoassay (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 &lt; 0.001). The odds ratio (OR) for mortality was 4.39 [95% confidence interval (CI) 2.10-9.16, p &lt; 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.},
  author       = {Hesselstrand, Roger and Scheja, Agneta and Wuttge, Dirk},
  issn         = {1502-7732},
  language     = {eng},
  pages        = {39--43},
  publisher    = {Taylor & Francis},
  series       = {Scandinavian Journal of Rheumatology},
  title        = {Scleroderma renal crisis in a Swedish systemic sclerosis cohort: survival, renal outcome, and RNA polymerase III antibodies as a risk factor.},
  url          = {http://dx.doi.org/10.3109/03009742.2011.610032},
  volume       = {41},
  year         = {2012},
}