Primary Biliary Cholangitis
GENERAL PRINCIPLES
Primary biliary cholangitis (PBC) is a cholestatic hepatic disorder of unknown etiology with autoimmune features characterized by a T lymphocyte-mediated attack on small intralobular bile ducts.
• PBC most often affects middle-aged women (gt;90%) and is more commonly described in Caucasians. It is caused by granulomatous destruction of the interlobular bile ducts, which leads to progressive ductopenia and cholestasis.
• Cholestasis is generally slowly progressive but can lead to cirrhosis and liver failure.
• Extrahepatic manifestations include keratoconjunctivitis sicca (Sjogren), renal tubular acidosis, gallstones, thyroid disease, scleroderma, Raynaud phenomenon, CREST syndrome (calcinosis, Raynaud phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia), and celiac disease.
DIAGNOSIS
Clinical Presentation
• Fatigue, jaundice, and pruritus are often the most troublesome symptoms.
• Patients may present de novo with manifestations of ESLD.
• Although there are no examination findings that are specific for PBC, xanthomata and xanthelasma can be a clue to underlying cholestasis.
Diagnostic Testing
• Antimitochondrial (AMA) antibodies are present in gt;90% of patients.
• Typical features include elevated levels of ALP, total bilirubin, cholesterol, and IgM.
• To confirm the diagnosis, two out of three of the following are required: ALP at least gt;1.5 the ULN, positive AMA (1:40 or higher), and histologic evidence of PBC.
DIAGNOSTIC PROCEDURES
Liver biopsy is helpful for both diagnosis and staging.
TREATMENT
Medications
• No curative therapy is available; treatment aims to slow down the progression of disease.
• Ursodeoxycholic acid (UDCA) (13-15 mg/kg/d PO) is a bile acid derivative with hepatocyte cytoprotective properties that stimulate hepatocellular and ductular secretions. Traditionally, UDCA has been suggested to reduce mortality when given over a long term.
• Obeticholic acid (OCA; 10-50 mg/d PO) is a derivative of the primary bile acid, chenodeoxycholic acid, and affects bile acid homeostasis. In a trial of patients with PBC with compensated liver disease who had an inadequate response to UDCA, 3-month treatment with OCA plus UDCA significantly reduced levels of ALP, GGT, and ALT when compared with UDCA plus placebo, but had a higher rate of pruritus.14
Surgical Management
• Liver transplantation is an option in advanced disease.
• Recurrent PBC after transplantation has been documented at a rate of 20% over 10 years.
Prognosis and Outcome
• PBC progresses along a path of increasingly severe histologic damage (florid bile duct lesions, ductular proliferation, fibrosis, and cirrhosis).
• Progression to cirrhosis and liver failure may occur years from diagnosis.
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