A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease

Halliday, John; Djordjević, Jelena; Lust, Mark; Culver, Emma L.; Braden, Barbara; Travis, Simon; Chapman, Roger W.

doi:10.1016/j.crohns.2011.07.015

Cited by 73 publications

(66 citation statements)

References 20 publications

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“…Lastly, the association of small-duct PSC appears to be stronger with Crohn’s colitis than with UC[8,66]. …”

Section: Variant Psc Syndromementioning

confidence: 99%

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Gidwaney¹,

Pawa²,

Das³

2017

WJG

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Primary sclerosing cholangitis (PSC) is a disease of the biliary tract, which has been documented in the literature since 1867. This disease has a strong predilection for affecting men and can be seen in individuals as young as 2 years of age. PSC has a strong associated with inflammatory bowel disease, more commonly with ulcerative colitis, and is also part of the clinical spectrum of IgG4-related diseases. Small-duct PSC, a variant of PSC, also has an association with inflammatory bowel disease. The exact pathogenesis of PSC is not well understood at present, however, is likely a combination of a genetic predisposition with alteration of the molecular structure of the gut. Abnormal serum liver chemistry and presence of certain autoimmune markers are usually the first indicators leading to a diagnosis of PCS, however, these may often be normal in early stages of this disease. The diagnosis is made by cholangiography, which is now considered the gold standard. PSC is a known pre-malignant condition. Such patients have an increased risk of developing cholangiocarcinoma, gallbladder neoplasia, and colon cancer. Many new treatment modalities have emerged in the recent past, including anti-tumor necrosis factor- α and anti-integrins; however, liver transplantation is the only known cure for PSC. Despite past and present research, PSC remains an enigmatic biliary disease with few viable treatment options.

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“…Lastly, the association of small-duct PSC appears to be stronger with Crohn’s colitis than with UC[8,66]. …”

Section: Variant Psc Syndromementioning

confidence: 99%

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Gidwaney¹,

Pawa²,

Das³

2017

WJG

View full text Add to dashboard Cite

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“…In a subgroup analysis of 80 PSC-IBD patients compared with 80 IBD controls, 85% of PSC-IBD patients had inflammation of the right hemicolon, compared with 54% of those with IBD alone. Interestingly, in contrast to PSC-UC, the clinical activity of Crohn's colitis in patients with PSC is similar to patients who have Crohn's colitis without PSC [24]. …”

Section: Ibd In Pscmentioning

confidence: 99%

Primary Sclerosing Cholangitis

Williamson

Chapman

2014

Dig Dis

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Primary sclerosing cholangitis (PSC) is a chronic, cholestatic liver disease caused by diffuse inflammation and fibrosis that can involve the entire biliary tree. It is a progressive disorder which can ultimately lead to biliary cirrhosis, portal hypertension and hepatic failure. PSC is a complex genetic disorder with male predominance. Environmental predisposing factors include non-smoking. It is closely associated with inflammatory bowel disease (IBD), particularly ulcerative colitis, which occurs in about two thirds of PSC cases. Recent studies have suggested that PSC-IBD is a separate disease entity from IBD alone with distinctive genetic and phenotypic characteristics. Most PSC patients are asymptomatic at presentation; clinical symptoms include fatigue, jaundice, weight loss, right upper quadrant pain and pruritis. Serum biochemical tests indicate cholestasis, and diagnosis is usually established by cholangiography. In symptomatic patients, median survival from presentation to death or liver transplantation is about 12 years. It is a premalignant condition, and the majority of deaths are from malignancy, particularly cholangiocarcinoma or colonic cancer. PSC has no curative treatment. Medical treatment with ursodeoxycholic acid may slow progression of the disease and reduce colonic dysplasia, though trials lack statistical significance. Liver transplantation is the only option in young patients with PSC and advanced liver disease.

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“…Cholangiocarcinoma however, does not seem to occur in patients with small-duct disease without progression to largeduct PSC [20]. The association of small-duct PSC appears to be stronger with Crohn's colitis than with UC [21,22], however as put by Jurgen Ludwig [23], the distinguished liver pathologist formerly at the Mayo Clinic: "No valid scientific reason can be found to suggest that small-duct PSC could not occur without large-duct PSC and IBD. Small-duct PSC may recur after liver transplantation [5].…”

Section: Discussionmentioning

confidence: 95%

Small Duct Sclerosing Cholangitis: A Case Report and Review of Literature

Wadea¹

2017

GHOA

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PSC often undiagnosed since approximately 40%-50% of patients with this disease are asymptomatic. Fatigue, fever, jaundice, pruritus, and vague upper abdominal discomfort are the most commonly described symptoms at the time of diagnosis. PSC involves both the intra-and extra-hepatic bile ducts in the majority of patients while an estimated 25% of patents only have isolated intra-hepatic involvement. In other reports intr-ahepatic involvement occur in only 10% of cases.

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A unique clinical phenotype of primary sclerosing cholangitis associated with Crohn's disease

Abstract: Unlike PSC/UC, patients with PSC/CD were as likely to be female as male, more commonly had small duct PSC and less commonly progressed to cancer, liver transplantation, or death. Compared to patients with isolated CD, patients with PSC/CD were less likely to smoke or have ileal disease.

Cited by 73 publications

References 20 publications

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Primary Sclerosing Cholangitis

Small Duct Sclerosing Cholangitis: A Case Report and Review of Literature

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