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Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis

Bergquist, Annika; Montgomery, Scott M.; Bahmanyar, Shahram; Olsson, Rolf; Danielsson, Ake; Lindgren, Stefan LU ; Prytz, Hanne LU ; Hultcrantz, Rolf; Loof, Lars and Sandberg-Gertzen, Hanna, et al. (2008) In Clinical Gastroenterology and Hepatology 6(8). p.939-943
Abstract
Background & Aims: The importance of genetic factors for the development of primary sclerosing cholangitis (PSC) is incompletely understood. This study assessed the risk of PSC and inflammatory bowel disease (IBD) among first-degree relatives of patients with PSC, compared with the first-degree relatives of a cohort without PSC. Methods: Subjects from the national Swedish cohort of PSC patients (n = 678) were matched for date of birth, sex, and region to up to 10 subjects without a diagnosis of PSC (n = 6347). Linkage through general population registers identified first-degree relatives of subjects in both the PSC and comparison cohorts (n = 34,092). Diagnoses among first-degree relatives were identified by using the Inpatient... (More)
Background & Aims: The importance of genetic factors for the development of primary sclerosing cholangitis (PSC) is incompletely understood. This study assessed the risk of PSC and inflammatory bowel disease (IBD) among first-degree relatives of patients with PSC, compared with the first-degree relatives of a cohort without PSC. Methods: Subjects from the national Swedish cohort of PSC patients (n = 678) were matched for date of birth, sex, and region to up to 10 subjects without a diagnosis of PSC (n = 6347). Linkage through general population registers identified first-degree relatives of subjects in both the PSC and comparison cohorts (n = 34,092). Diagnoses among first-degree relatives were identified by using the Inpatient Register. Results: The risk of cholangitis was statistically significantly increased in offspring, siblings, and parents of the PSC patient cohort, compared with relatives of the comparison cohort, with the hazard ratios and 95% confidence intervals, 11.5 (1.6-84.4), 11.1 (3.3-37.8), and 2.3 (0.9-6.1), respectively. The hazard ratios for ulcerative colitis (UC) among first-degree relatives of all PSC patients was 3.3 (2.3-4.9) and for Crohn's disease 1.4 (0.8-2.5). The risk of UC for relatives of PSC patients without IBD was also increased, 7.4 (2.9-18.9). Conclusions: First-degree relatives of patients with PSC run an increased risk of PSC, indicating the importance of genetic factors in the etiology of PSC. First-degree relatives of PSC patients without IBD are also at an increased risk of UC, which might indicate shared genetic susceptibility factors for PSC and UC. (Less)
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type
Contribution to journal
publication status
published
subject
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Clinical Gastroenterology and Hepatology
volume
6
issue
8
pages
939 - 943
publisher
Elsevier
external identifiers
  • wos:000258292500020
  • scopus:47949124659
ISSN
1542-7714
DOI
10.1016/j.cgh.2008.03.016
language
English
LU publication?
yes
id
bb0bfcf4-4932-4926-b3ac-7b55d40fc7ff (old id 1253492)
date added to LUP
2008-11-14 14:51:05
date last changed
2017-09-24 03:39:04
@article{bb0bfcf4-4932-4926-b3ac-7b55d40fc7ff,
  abstract     = {Background & Aims: The importance of genetic factors for the development of primary sclerosing cholangitis (PSC) is incompletely understood. This study assessed the risk of PSC and inflammatory bowel disease (IBD) among first-degree relatives of patients with PSC, compared with the first-degree relatives of a cohort without PSC. Methods: Subjects from the national Swedish cohort of PSC patients (n = 678) were matched for date of birth, sex, and region to up to 10 subjects without a diagnosis of PSC (n = 6347). Linkage through general population registers identified first-degree relatives of subjects in both the PSC and comparison cohorts (n = 34,092). Diagnoses among first-degree relatives were identified by using the Inpatient Register. Results: The risk of cholangitis was statistically significantly increased in offspring, siblings, and parents of the PSC patient cohort, compared with relatives of the comparison cohort, with the hazard ratios and 95% confidence intervals, 11.5 (1.6-84.4), 11.1 (3.3-37.8), and 2.3 (0.9-6.1), respectively. The hazard ratios for ulcerative colitis (UC) among first-degree relatives of all PSC patients was 3.3 (2.3-4.9) and for Crohn's disease 1.4 (0.8-2.5). The risk of UC for relatives of PSC patients without IBD was also increased, 7.4 (2.9-18.9). Conclusions: First-degree relatives of patients with PSC run an increased risk of PSC, indicating the importance of genetic factors in the etiology of PSC. First-degree relatives of PSC patients without IBD are also at an increased risk of UC, which might indicate shared genetic susceptibility factors for PSC and UC.},
  author       = {Bergquist, Annika and Montgomery, Scott M. and Bahmanyar, Shahram and Olsson, Rolf and Danielsson, Ake and Lindgren, Stefan and Prytz, Hanne and Hultcrantz, Rolf and Loof, Lars and Sandberg-Gertzen, Hanna and Almer, Sven and Askling, Johan and Ehlin, Anna and Ekbom, Anders},
  issn         = {1542-7714},
  language     = {eng},
  number       = {8},
  pages        = {939--943},
  publisher    = {Elsevier},
  series       = {Clinical Gastroenterology and Hepatology},
  title        = {Increased risk of primary sclerosing cholangitis and ulcerative colitis in first-degree relatives of patients with primary sclerosing cholangitis},
  url          = {http://dx.doi.org/10.1016/j.cgh.2008.03.016},
  volume       = {6},
  year         = {2008},
}