Cirrhosis causes
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] ; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]
Overview
Causes
Common Causes
Cirrhosis has many possible causes; sometimes more than one cause is present in the same patient. In the Western World, chronic alcoholism and hepatitis C are the most common causes.
- Alcoholic liver disease (ALD) Alcohol seems to injure the liver by blocking the normal metabolism of protein, fats, and carbohydrates. Patients may also have concurrent alcoholic hepatitis with fever, hepatomegaly, jaundice, and anorexia. AST and ALT are both elevated but less than 300 IU/L with a AST:ALT ratio > 2.0, a value rarely seen in other liver diseases. Liver biopsy may show hepatocyte necrosis, Mallory bodies, neutrophilic infiltration with perivenular inflammation.
- Chronic hepatitis C. Infection with this virus causes inflammation of and low grade damage to the liver that over several decades can lead to cirrhosis. Can be diagnosed with serologic assays that detect hepatitis C antibody or viral RNA. The enzyme immunoassay, EIA-2, is the most commonly used screening test in the US.
- Chronic hepatitis B. The hepatitis B virus is probably the most common cause of cirrhosis worldwide, especially South-East Asia, but it is less common in the United States and the Western world. Hepatitis B causes liver inflammation and injury that over several decades can lead to cirrhosis. Hepatitis D is dependent on the presence of hepatitis B, but accelerates cirrhosis in co-infection. Chronic hepatitis B can be diagnosed with detection of HBsAG > 6 months after initial infection. HBeAG and HBV DNA are determined to assess whether patient will need antiviral therapy.
- Non-alcoholic steatohepatitis (NASH). In NASH, fat builds up in the liver and eventually causes scar tissue. This type of hepatitis appears to be associated with diabetes, protein malnutrition, obesity, coronary artery disease, and treatment with corticosteroid medications. This disorder is similar to that of alcoholic liver disease but patient does not have an alcohol history. Biopsy is needed for diagnosis.
- Primary biliary cirrhosis. May be asymptomatic or complain of fatigue, pruritus, and non-jaundice skin hyperpigmentation with hepatomegaly. There is prominent alkaline phosphatase elevation as well as elevations in cholesterol and bilirubin. Gold standard diagnosis is antimitochondrial antibodies with liver biopsy as confirmation if showing florid bile duct lesions. It is more common in women.
- Primary sclerosing cholangitis. PSC is a progressive cholestatic disorder presenting with pruritus, steatorrhea, fat soluble vitamin deficiencies, and metabolic bone disease. There is a strong association with inflammatory bowel disease (IBD), especially ulcerative colitis. Diagnosis is best with contrast cholangiography showing diffuse, multifocal strictures and focal dilation of bile ducts, leading to a beaded appearance. Non-specific serum immunoglobulins may also be elevated.
- Autoimmune hepatitis. This disease is caused by the immunologic damage to the liver causing inflammation and eventually scarring and cirrhosis. Findings include elevations in serum globulins, especially gamma globulins. Therapy with prednisone +/-azathioprine is beneficial. Cirrhosis due to autoimmune hepatitis still has 10-year survival of 90%+. There is no specific tool to diagnose autoimmune but it can be beneficial to initiate a trial of corticosteroids.
- Hereditary hemochromatosis. Usually presents with family history of cirrhosis, skin hyperpigmentation, diabetes mellitus, pseudogout, and/or cardiomyopathy, all due to signs of iron overload. Labs will show fasting transferrin saturation of > 60% and ferritin > 300 ng/mL. Genetic testing may be used to identify HFE mutations. If these are present, biopsy may not need to be performed. Treatment is with phlebotomy to lower total body iron levels.
- Wilson's disease. Autosomal recessive disorder characterized by low serum ceruloplasmin and increased hepatic copper content on liver biopsy. May also have Kayser-Fleischer rings in the cornea and altered mental status.
- Alpha 1-antitrypsin deficiency (AAT). Autosomal recessive disorder. Patients may also have COPD, especially if they have a history of tobacco smoking. Serum AAT levels are low. Recombinant AAT is used to prevent lung disease due to AAT deficiency.
- Cardiac cirrhosis. Due to chronic right sided heart failure which leads to liver congestion.
- Galactosemia
- Glycogen storage disease type IV
- Cystic fibrosis
- Drugs or toxins
- Certain parasitic infections (such as schistosomiasis)
Causes by Organ System
Causes in Alphabetical Order [1] [2]
- Abetalipoproteinemia
- Alagille syndrome
- Alcoholic liver disease
- Alpers disease
- Alpha 1-antitrypsin deficiency
- Alström syndrome
- Autoimmune cholangiopathy
- Autoimmune hepatitis
- Berardinelli lipodystrophy syndrome
- Bile duct stricture
- Biliary atresia
- Budd-Chiari Syndrome
- Carbohydrate deficient glycoprotein syndrome type 1a
- Cardiac cirrhosis
- Right sided cardiac failure
- Caroli disease
- Cerebrohepatorenal syndrome
- Cholestasis-oedema syndrome, Norwegian type
- Cholesterol ester storage disease
- Congenital hepatic fibrosis
- Constrictive pericarditis
- Cor Pulmonale
- Cruveilhier-Baumgarten syndrome
- Cystic fibrosis
- Ethanol
- Fasciola hepatica
- Galactosemia
- Glycogen storage disease type IV
- Graft versus host disease
- Granulomatous cirrhosis
- Haemosiderosis
- Hemochromatosis
- Hepatic vein thrombosis
- Hepatitis B
- Hepatitis C
- Hereditary fructose intolerance
- Hypervitaminosis A
- Indian familial childhood cirrhosis
- Isoniazid
- Keratitis-ichthyosis-deafness syndrome, autosomal recessive
- Methotrexate
- Methyldopa
- Non-alcoholic steatohepatitis
- Parenteral nutrition
- Polycystic kidney disease, autosomal recessive
- Porphyria cutanea tarda type 2 (familial)
- Primary biliary cirrhosis
- Primary sclerosing cholangitis
- Sarcoidosis
- Schistosomiasis
- Sickle Cell Disease
- Sickle cell disease
- Steatohepatitis
- Thalassemia
- Tricuspid insufficiency
- Tyrosinaemia type 1
- Visceral leishmaniasis
- Wilson disease