Primary Sclerosing Cholangitis With Features of Autoimmune Hepatitis: Exploring the Global Variation in Management. Issue 3 (16th January 2020)

• Record Type: Journal Article
• Authors: Milkiewicz, Piotr; Krawczyk, Marcin; Wunsch, Ewa; Ponsioen, Cyriel; Hirschfield, Gideon M.; Hubscher, Stefan G.
• Is Part Of: Hepatology communications. Volume 4:Issue 3(2020)
• Page Start: 399
• Page End: 408
• Publication Date: 2020-01-16
• Subjects: Hepatology -- Periodicals; Liver -- Diseases -- Periodicals; Liver Diseases; Gastroenterology; Periodicals; Fulltext; Internet Resources; Periodicals; 616.36
• DOI: 10.1002/hep4.1467
• Languages: English
• ISSNs: 2471-254X

Abstract

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 frequently than their counterparts in North America ( P < 0.05 in three out of four cases), who advocated the use of immunosuppression alone more commonly than Europeans ( P = 0.005 in case 2). Conclusions: We document a wide variation in clinical decision making in the context of managing patients with a potential AIH/PSC variant. Guidance, likely based on systematic studies arising from prospective registries, is needed to better address this difficult clinician problem. Abstract : This study is a survey performed across a group of 80 hepatologists from 23 countries/five continents on their management of patients with potential AIH/PSC; it very clearly illustrates a global confusion to the treatment of this difficult clinical problem. We document a wide variability in clinical decision making among experts, additionally showing a significant difference between North American and European hepatologists in terms of their therapeutic approaches.