Small-duct primary sclerosing cholangitis

Bjõrnsson, Einar

doi:10.1007/s11894-009-0006-6

Cited by 29 publications

(22 citation statements)

References 34 publications

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“…Crohn's disease is more commonly observed compared with largeduct PSC [51]. PSC-AIH overlap also appears to be more frequent (27%) among patients with small-duct PSC compared with large-duct PSC (11%) [34].…”

Section: Small-duct Pscmentioning

confidence: 98%

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Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Zein

Lindor

2010

Curr Gastroenterol Rep

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Primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) are the two most common causes of chronic cholestatic liver disease in adults. In PBC, therapy with ursodeoxycholic acid (UDCA) is safe and has been associated with tangible biochemical, histologic, and survival benefits. However, a need for different or adjuvant therapies remains for specific subsets of PBC patients, including those who do not respond to UDCA and those who have advanced histologic disease at presentation. Similarly, beneficial therapies for disease-related symptoms that do not typically respond to UDCA (eg, fatigue and pruritus) are still needed. In contrast to PBC, no medical therapy of proven benefit has been identified for patients with PSC. In PBC and PSC, adequate management of complications of chronic cholestasis is important. For both diseases, liver transplantation is the only curative option.

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Section: Small-duct Pscmentioning

confidence: 98%

“…Liver biopsy may provide staging information, but is often nonspecific in PSC and false-negatives often occur. Nevertheless, a liver biopsy is important in the evaluation of patients who may have small-duct PSC [34].…”

Section: Clinical Presentation and Diagnosismentioning

confidence: 99%

Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Zein

Lindor

2010

Curr Gastroenterol Rep

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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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“…For those patients who had not undergone liver biopsy, the diagnosis was deemed unlikely by the absence of the classic triad for AMA negative PBC: high serum cholesterol, ceruloplasmin and IgM levels [2]. Regarding SD-PSC, the absence of concomitant IBD, negative MRCP findings and lack of concentric fibrosis of bile ducts on liver biopsy argued against that condition [3]. Autoimmune cholangitis was excluded if serum AST values were not low, ANA titres not high and cholangitis not present on liver biopsy [4].…”

Section: Discussionmentioning

confidence: 99%

Adult idiopathic cholestasis: a condition more common in the Canadian Inuit?

Minuk

Pollock

Uhanova

2017

International Journal of Circumpolar Health

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Despite extensive investigations, some patients have no identifiable cause for their cholestatic liver enzyme abnormalities. The aim of this study was to document the clinical, laboratory, radiologic and histologic features of adult patients with idiopathic cholestasis (AIC). A computerised database of referred patients to a tertiary care hospital outpatient department for assessment of hepatobiliary disorders between 2005 and 2015 was employed to identify and describe features associated with AIC. Of 6,560 patient referrals, sufficient documentation to warrant a diagnosis of AIC was present in 17 (0.26%) cases. Of the 17, a disproportionate number were Canadian Inuit (7/60, 12% Inuit referrals vs. 10/6,500, 0.16% non-Inuit referrals, p<0.0001). The median age of the 17 subjects was 57 years and nine (53%) were female. Clinical and/or laboratory evidence of autoimmune disorders was present in six (35%) cases. Clinical features of hepatic decompensation, radiologic findings in keeping with cirrhosis and histologic confirmation of cirrhosis were present in 47%, 31% and 42% of individuals, respectively. There were no significant improvements in cholestatic liver enzymes and function tests in those treated with ursodiol and/or immunomodulants (n=7) compared to those left untreated (n=10). In conclusion, AIC is a rare condition diagnosed by exclusion. It appears to be more common in the Canadian Inuit population and those with autoimmune disorders. Advanced liver disease is a frequent finding at presentation. Intervention with ursodiol and/or immunomodulants does not appear to be of therapeutic value.

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Small-duct primary sclerosing cholangitis

Cited by 29 publications

References 34 publications

Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Latest and Emerging Therapies for Primary Biliary Cirrhosis and Primary Sclerosing Cholangitis

Pathogenesis and clinical spectrum of primary sclerosing cholangitis

Adult idiopathic cholestasis: a condition more common in the Canadian Inuit?

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