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

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.

MARC1 p.A165T variant is associated with decreased markers of liver injury and enhanced antioxidant capacity in autoimmune hepatitis

“…Our center serves as a referral unit for patients with AIH in Poland, which might be one of the reasons why our cohort was characterized by a high prevalence of AIH-PSC and AIH-PBC overlaps with a relatively high number of patients with cirrhosis. These variants of AIH are typically more difficult to treat, with a wide variation in clinical decisions together with these patients, even in referral centers 37 .…”

EASL Clinical Practice Guidelines on sclerosing cholangitis

“…58 Substantial variation exists with respect to the diagnosis and management of PSC with co-existing features of AIH. 59 This variation arises from 2 main caveats: first, PSC and AIH share several core features and, second, there is no reliable serum biomarker to diagnose either condition. Hypergammaglobulinemia, presence of various autoantibodies and elevation of serum transaminases can be seen both in AIH and in PSC.…”

Autoimuninių Kepenų Ligų Persidengimo Sindromai: Diagnostika Ir Gydymo Galimybės

“…Apklausus 80 gydytojų hepatologų, dauguma respondentų AIH-PSC pacientus gydytų kombinuota terapija. Populiariausia gydymo taktika buvo prednizolono (iki 30 mg per parą) ir azatioprino (iki 2 mg/kg per parą) derinys su UDCR (13-15 mg/kg per parą) [29]. Remiantis 2022 m. EASL gairėmis, pacientams, sergantiems PSC, turintiems biocheminių ir histologinių AIH požymių, rekomenduojama imunosupresinio gydymo galimybė predniz(ol)ono ir azatrioprino deriniu [5,30].…”