Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management

Hepatology Communications
Piotr MilkiewiczStefan G Hubscher

Abstract

Patients with primary sclerosing cholangitis (PSC) frequently manifest features of autoimmune hepatitis (AIH). We sought to understand factors affecting expert management, with the goal of facilitating uniformity of care. A Survey Monkey questionnaire with four hypothetical cases suggesting a potential AIH/PSC variant was sent to hepatologists spanning global practices. Eighty responses from clinicians in 23 countries were obtained. Most of the respondents would request a liver biopsy, and stated that the cases presented could not be appropriately managed without a biopsy. Despite the fact that histology did not unequivocally support an AIH/PSC variant in three of the four cases, this diagnosis was reached by most of the respondents for all cases, except case 1, in which 49% were diagnosed with AIH/PSC. There was a wide variation of suggested medical treatment. For three cases, the most commonly chosen treatment options did not exceed 35%, indicating a lack management consensus. Most respondents would treat with ursodeoxycholic acid, despite current American Association for the Study of Liver Diseases guidelines, either alone or in combination with immunosuppression. European clinicians recommended ursodeoxycholic acid more fre...Continue Reading

References

Jun 10, 2008·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Elke M HennesUNKNOWN International Autoimmune Hepatitis Group
Jul 9, 2008·World Journal of Gastroenterology : WJG·Johannes-Roksund HovTom-H Karlsen
Jun 9, 2009·Journal of Hepatology·UNKNOWN European Association for the Study of the Liver
Jul 4, 2009·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Maïté LewinChristophe Corpechot
Jan 27, 2010·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Roger ChapmanUNKNOWN American Association for the Study of Liver Diseases
Nov 12, 2010·Journal of Hepatology·Kirsten Muri BobergUNKNOWN International Autoimmune Hepatitis Group
Jul 3, 2013·Lancet·Gideon M HirschfieldDavid H Adams
Sep 6, 2015·Journal of Hepatology·UNKNOWN European Association for the Study of the Liver
Jan 16, 2016·International Journal of Hepatology·Hugo PerazzoRodolfo Castro
Sep 18, 2016·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Salvatore PettaVictor de Ledinghen
Dec 23, 2016·Alimentary Pharmacology & Therapeutics·R LiberalUNKNOWN International Autoimmune Hepatitis Group (IAIHG)
Aug 31, 2017·Scandinavian Journal of Gastroenterology·Marcin KrawczykPiotr Milkiewicz

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Methods Mentioned

BETA
biopsy
biopsies

Software Mentioned

Survey Monkey
Excel
FibroScan

Related Concepts

Related Feeds

Autoimmune Hepatitis

Autoimmune hepatitis formerly called lupoid hepatitis, is a chronic, autoimmune disease of the liver that occurs when the body's immune system attacks liver cells causing the liver to be inflamed. Discover the latest research on autoimmune hepatitis here.

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