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The Many Faces of Primary Sclerosing Cholangitis: Controversy Abounds

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Abstract

Primary sclerosing cholangitis (PSC) is notoriously challenging to manage given its heterogeneity with regard to diagnosis, management, and progression. The lack of disease-modifying therapy and variable rate of onset of cirrhosis, portal hypertension-related decompensating events, jaundice, pruritus, biliary complications, and need for liver transplantation is deeply unsettling to clinicians and patients alike. Recent updated practice guidance by the American Association for the Study of Liver Diseases and the European Association for the Study of the Liver endeavored to highlight some of these challenges. However, these references only briefly address clinical dilemmas that providers face on a daily basis. This review aims to further discuss these controversial topics, including providing insight into the utility of ursodeoxycolic acid, the significance of alkaline phosphatase normalization, when to consider PSC variants and mimickers, and the implications of continuous hepatobiliary malignancy screening. In particular, there has been a growing body of literature raising concern about repeat exposure to gadolinium-containing contrast. Patients with PSC are potentially at risk for large lifetime exposure to gadolinium related to frequent magnetic resonance imaging scans and whether this carries any negative long-term adverse effects remains unknown.

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Abbreviations

AASLD:

American Association for the Study of Liver Diseases

ALP:

Alkaline phosphatase

AIH:

Autoimmune hepatitis

ANA:

Antinuclear antibody

ASMA:

Anti-smooth muscle antibody

CA 19–9:

Carbohydrate antigen 19–9

CCA:

Cholangiocarcinoma

DN:

Dentate nucleus

EASL:

European Association for the Study of the Liver

GP:

Globus pallidus

IAIHG:

International Autoimmune Hepatitis Group

IgG4-RD:

IgG4-related disease

IRC:

IgG4-related cholangiopathy

MRCP:

Magnetic resonance cholangiopancreatography

PBC:

Primary biliary cholangitis

PSC:

Primary sclerosing cholangitis

SSC:

Secondary sclerosing cholangitis

UDCA:

Ursodeoxycholic acid

ULN:

Upper limit of normal

US:

Ultrasound

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Funding

BJW has received research support through the AASLD Foundation Clinical, Translational and Outcomes Research Award (50031). No assistance was received with regard to manuscript preparation.

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  1. Division of Gastroenterology and Hepatology, School of Medicine, University of Virginia, PO Box 800708, Charlottesville, VA, 22908, USA

    Brian J. Wentworth & Stephen H. Caldwell

  2. Department of Radiology and Medical Imaging, University of Virginia Health System, Charlottesville, VA, USA

    Rachita Khot

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BJW: conceptualization, acquisition of data, drafting of manuscript, revision of manuscript, final approval, RK: acquisition of data, revision of manuscript, final approval, SHC: conceptualization, acquisition of data, revision of manuscript, final approval.

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Wentworth, B.J., Khot, R. & Caldwell, S.H. The Many Faces of Primary Sclerosing Cholangitis: Controversy Abounds. Dig Dis Sci 68, 3514–3526 (2023). https://doi.org/10.1007/s10620-023-08003-2

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