Primary biliary cholangitis - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Primary biliary cholangitis (PBC) is characterized by progressive intrahepatic bile duct damage and loss.
PBC is significantly more common in women than in men. Peak incidence is around age 40 years, and median age at diagnosis is 65 years.
The combination of a cholestatic pattern of serum liver tests (elevated alkaline phosphatase, gamma-glutamyl transferase, or both) and a PBC-specific autoantibody (typically antimitochondrial antibody) is sufficient for diagnosis in most patients, with no need for biopsy confirmation.
PBC is progressive in most patients; although, in many people, the rate of progression can be so slow that it may not be clinically relevant. Cirrhosis and its typical complications arise in the end stage.
Symptoms (typically pruritus and fatigue) can significantly lower the quality of life, even in patients with a very slowly progressive disease. These symptoms warrant treatment in their own right using specific regimens.

Characteristic autoantibody patterns in primary biliary cholangitis. White arrow: antimitochondrial staining; red arrow: multiple nuclear dot ANA staining

From the collection of DEJ Jones; used with permission.

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Definition

Epidemiology

Etiology

Pathophysiology

Images

Characteristic autoantibody patterns in primary biliary cholangitis. White arrow: antimitochondrial staining; red arrow: multiple nuclear dot ANA staining
Characteristic histologic appearances of primary biliary cholangitis: (a) early-stage disease; (b) advanced-stage disease; (c) disease with a significant inflammatory component

Citations

Key Articles

Lindor KD, Bowlus CL, Boyer J, et al. Primary biliary cholangitis: 2018 practice guidance from the American Association for the Study of Liver Diseases. Hepatology. 2019 Jan;69(1):394-419.[Abstract][Full Text]
European Association for the Study of the Liver. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017 Jul;67(1):145-72.[Abstract]
Nevens F, Andreone P, Mazzella G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med. 2016 Aug 18;375(7):631-43.[Abstract]

Referenced Articles

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