Internal Diseases and Therapy · MU Sofia

98. Primary biliary cholangitis (Primary biliary cirrhosis ; PBC). Primary sclerosing

Covers primary biliary cholangitis, an autoimmune disease destroying small liver bile ducts, with its symptoms, causes linked to anti-mitochondrial antibodies and associated autoimmune conditions, diagnosis including biopsy findings, UDCA and other treatments, and prognosis. It then describes primary sclerosing cholangitis, its link to inflammatory bowel disease, diagnostic criteria based on two of three findings, and its supportive management and prognosis before transplant.

Inside: 1 section · 15 flashcards · 3 min

Topic 99 of 139 in the MU Sofia syllabus for Internal Diseases and Therapy (Medicine, Years 4 and 5). The free AceMed note covers it in 1 section, about 481 words (3 min of reading), with 15 flashcards.

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  1. Primary biliary cirrhosis (PBC). Primary sclerosing cholangitis

Primary biliary cirrhosis (PBC). Primary sclerosing cholangitis

Primary biliary cholangitis (previously, primary biliary cirrhosis)

An autoimmune disease of the liver with progressive destruction of small bile ducts of the liver, leading to bile and toxin build up in the liver, causing cholestasis. This can eventually lead to fibrosis and cirrhosis.

Signs and symptoms

  • Fatigue is the most common, dry skin, dry eyes and itching
  • Possible jaundice
  • Decreased bone density and xanthomas
  • Can then lead to liver cirrhosis

Causes

  • Autoimmune, >90% of patients have anti-mitochondrial bodies (AMAs) against pyruvate dehydrogenase complex
  • Most important diagnostic lab indicator
  • Often comes with other autoimmune diseases (e.g. sjogren’s, rheumatoid arthritis, lupus

Diagnosis

  • Main differentials are with AI hepatitis and primary sclerosing cholangitis (below)
  • Elevated ASTs and ALTs along with ALP and increased bilirubin in advanced cases
  • AMAs are the most characteristic serological marker
  • Other autoantibodies may also be present (e.g. antinuclear ABs, anti-glycoprotein ABs and anti-centromere ABs)
  • Biopsy can also help identify the disease, the histopathologic findings are
  • Inflamed bile ducts with intraepithelial lymphocytes
  • Periductal epithelioid granulomas

Treatment

  • No known cure, medication may slow progression
  • Ursodeoxycholic acid (UDCA) helps reduce cholestasis and improves LFTs.
  • Cholestyramine helps absorb bile acids in gut rather than allowing them to be absorbed in the blood and therefore relieves symptoms; stanozolol, naltrexone and rifampicin relieve itching by other mechanisms
  • Can give vitamin A, D, E and K to supplement the low absorption of these lipid dependent vitamins

Prognosis - UDCA responders have near-normal survival; untreated patients who become jaundiced (bilirubin >2 mg/dL) have a mean survival time of 4 years, although liver transplants increase this.

Primary sclerosing cholangitis

This is a long term progressive disease of the liver and bile ducts, characterised by inflammation and scarring of bile ducts. As with PBC, this can eventually lead to cirrhosis and liver failure. PSC increases the risk of HCC, cholangiocarcinoma, colorectal cancer and others. 75% of patients also have iBD, most commonly ulcerative colitis.

Signs and symptoms

  • Half do not have symptoms
  • Those that do have symptoms have as a result of obstructive jaundice

Pathophysiology

  • Disease is autoimmune, with a strong relationship between PSC and human leukocyte antigen alleles
  • Inflammation of the bile ducts (cholangitis) causes narrowing and sclerosis of the ducts. This obstructed bile flow (cholestasis) which causes liver injury
  • As bile helps with absorption of lipid soluble vitamins, we also see malabsorption here (vitamin A, D, E and K)

Diagnosis

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