Risk factors in primary sclerosing cholangitis

Schrumpf, E.; Abdelnoor, Michel; Fausa, O; Elgjo, Kjell; Jenssen, E.; Kolmannskog, F.

doi:10.1016/s0168-8278(05)80618-x

Cited by 113 publications

(70 citation statements)

References 17 publications

(1 reference statement)

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“…Up to 20% of the PSC patients develop cholangiocarcinoma, and a high proportion (20%-80%, depending on ethnic group) of the patients have concomitant inflammatory bowel disease (IBD). [1][2][3] The etiology and pathogenesis of the disease are unknown, but both immunologic and genetic factors have been implicated. 4 Several studies have shown that susceptibility to develop PSC is associated with genes in the HLA complex.…”

mentioning

confidence: 99%

Primary sclerosing cholangitis is associated to an extended B8-DR3 haplotype including particular MICA and MICB alleles

WIENCKE¹,

SPURKLAND²,

SCHRUMPF³

et al. 2001

Hepatology

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Susceptibility to primary sclerosing cholangitis (PSC) is associated with HLA-B8, -DR3, -DR2, and -DR6. It is not established whether these HLA genes or closely linked genes confer the primary disease susceptibility. MICA and MICB genes are found in the class I region between HLA-B and DRB. MICA is expressed in gastrointestinal epithelium and activates ␥␦ T cells in the gut. Because PSC is strongly associated with inflammatory bowel disease, we investigated whether MICA and MICB contribute to the HLA-associated genetic susceptibility to develop PSC. The study included 130 PSC patients and 306 healthy controls, previously typed for HLA class I and II genes, typed for 5 MICA and 15 MICB microsatellite alleles. The phenotype frequencies of MICA5.1 and MICB24 were significantly increased among PSC patients compared with controls (90% vs. 74%; odds ratio [OR] ‫؍‬ 3.2; P c ‫؍‬ 3 ؋ 10 ؊3 and 58% vs. 29%; OR ‫؍‬ 3.3; P c < 1 ؋ 10 ؊7 , respectively). When stratified for B8-or DR3-positive and -negative individuals, the association of these markers to PSC was no longer evident. However, we observed that B8 and DR3 were associated to PSC only in the presence of both MICA5.1 and MICB24 markers. The frequency of individuals carrying all 4 alleles was significantly increased among the PSC patients compared with controls (49% vs. 18%; OR ‫؍‬ 4.5; P c < 1 ؋ 10 ؊7 ). Our data indicate that PSC is associated to the extended B8-MICA5.1-MICB24-DR3 haplotype. (HEPATOLOGY 2001;34:625-630.)Primary sclerosing cholangitis (PSC) is a chronic liver disease characterized by inflammatory and fibrotic bile duct lesions forming multiple strictures and ectatic dilatations of the intra-and extrahepatic bile ducts.

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mentioning

confidence: 99%

Primary sclerosing cholangitis is associated to an extended B8-DR3 haplotype including particular MICA and MICB alleles

WIENCKE¹,

SPURKLAND²,

SCHRUMPF³

et al. 2001

Hepatology

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“…Seventy percent to 100% of PSC patients are reported to have concomitant IBD. [1][2][3] Patients with PSC and ulcerative colitis (UC) do not only have an increased risk of developing colonic dysplasia and colonic cancer, 4,5 but do have an increased risk of developing cholangiocarcinoma (CC). [6][7][8] In addition, PSC patients with colonic dysplasia/cancer seem to be more prone to develop CC.…”

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Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: A case-control study

et al. 1998

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“…It predisposes to the development of cholangiocarcinoma (CC). From selected cohorts of PSC patients [1][2][3][4][5] with a total number of 536 patients, the average prevalence of this neoplasm was calculated to be 9%. The time from the PSC diagnosis until recognition of CC ranged from 1 to 25 years.…”

mentioning

confidence: 99%

Detection of cholangiocarcinoma in primary sclerosing cholangitis by positron emission tomography

et al. 1998

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Risk factors in primary sclerosing cholangitis

Cited by 113 publications

References 17 publications

Primary sclerosing cholangitis is associated to an extended B8-DR3 haplotype including particular MICA and MICB alleles

Primary sclerosing cholangitis is associated to an extended B8-DR3 haplotype including particular MICA and MICB alleles

Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: A case-control study

Detection of cholangiocarcinoma in primary sclerosing cholangitis by positron emission tomography

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