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]

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

Cardiovascular	Cardiac cirrhosis, Right sided cardiac failure,Constrictive pericarditis, Cor Pulmonale, Tricuspid insufficiency
Chemical / poisoning	Aflatoxin
Dermatologic	Keratitis-ichthyosis-deafness syndrome, Addison-Gull syndrome , Reynolds syndrome , Tricho-hepato-enteric syndrome
Drug Side Effect	Amiodarone, Ethanol, Isoniazid, Methotrexate, Methyldopa
Ear Nose Throat	No underlying causes
Endocrine	No underlying causes
Environmental	No underlying causes
Gastroenterologic	Addison-Gull syndrome , Alpha 1-antitrypsin deficiency , Alström syndrome, Autoimmune cholangiopathy, Autoimmune hepatitis, Bearn-Kunkel syndrome , Bile duct stricture, Biliary atresia, Budd-Chiari Syndrome, Caroli disease, Cerebrohepatorenal syndrome, Ceroid storage disease, Cholestasis-oedema syndrome, Norwegian type, Cruveilhier-Baumgarten syndrome, Congenital hepatic fibrosis, Cystic fibrosis, Granulomatous cirrhosis, Hemochromatosis, Hepatic vein thrombosis, Hereditary fructose intolerance,Indian familial childhood cirrhosis, Non-alcoholic steatohepatitis , Primary biliary cirrhosis, Primary sclerosing cholangitis, Reynolds syndrome , Tricho-hepato-enteric syndrome , Wilson disease
Genetic	Abetalipoproteinemia, Alagille syndrome, Alpers disease, Alpha 1-antitrypsin deficiency ,Alström syndrome, Berardinelli lipodystrophy syndrome, Carbohydrate deficient glycoprotein syndrome type 1a,Carbohydrate-deficient glycoprotein syndrome type 1b, Caroli disease, Ceroid storage disease , Cholestasis-oedema syndrome, Norwegian type,Congenital hepatic fibrosis, Cystic fibrosis, Fanconi disease, Fructose-1-phosphate aldolase deficiency, Galactosemia, Glycogen storage disease type IV, Haemosiderosis,Hemochromatosis, Hereditary fructose intolerance, Keratitis-ichthyosis-deafness syndrome, autosomal recessive,Polycystic kidney disease, autosomal recessive, Porphyria cutanea tarda type 2 (familial), Sickle cell disease,Tyrosinaemia type 1, Wilson disease
Hematologic	Budd-Chiari Syndrome, Erythropoietic protoporphyria, Fanconi disease, Haemosiderosis, Hepatic vein thrombosis, Porphyria cutanea tarda type 2 (familial), Sickle cell disease, Thalassemia, Tyrosinaemia type 1
Iatrogenic	Bile duct stricture, Graft versus host disease, Parenteral nutrition
Infectious Disease	Fasciola hepatica, Hepatitis B, Hepatitis C, Schistosoma haematobium, Schistosoma japonicum,Schistosoma mansoni, Visceral leishmaniasis
Musculoskeletal / Ortho	No underlying causes
Neurologic	Alpers disease, Cerebrohepatorenal syndrome, Keratitis-ichthyosis-deafness syndrome, autosomal recessive
Nutritional / Metabolic	Abetalipoproteinemia, Carbohydrate deficient glycoprotein syndrome type 1a, Carbohydrate-deficient glycoprotein syndrome type 1b, Ceroid storage disease , Cholesterol ester storage disease, Fructose-1-phosphate aldolase deficiency, Galactosemia, Glycogen storage disease type IV,Hypervitaminosis A, Parenteral nutrition
Obstetric/Gynecologic	No underlying causes
Oncologic	No underlying causes
Opthalmologic	Keratitis-ichthyosis-deafness syndrome, autosomal recessive
Overdose / Toxicity	Acetaminophen overdose
Psychiatric	No underlying causes
Pulmonary	No underlying causes
Renal / Electrolyte	Cerebrohepatorenal syndrome, Fanconi disease, Polycystic kidney disease, autosomal recessive
Rheum / Immune / Allergy	Autoimmune cholangiopathy, Autoimmune hepatitis, Graft versus host disease, Primary biliary cirrhosis, Primary sclerosing cholangitis, Reynolds syndrome , Sarcoidosis
Sexual	No underlying causes
Trauma	No underlying causes
Urologic	No underlying causes
Dental	No underlying causes
Miscellaneous	Alcoholic liver disease

Causes in Alphabetical Order

• Abetalipoproteinemia
• Acetaminophen overdose
• Addison-Gull syndrome
• Aflatoxin
• Alagille syndrome
• Alcoholic liver disease
• Alpers disease
• Alpha 1-antitrypsin deficiency
• Alström syndrome
• Amiodarone
• Autoimmune cholangiopathy
• Autoimmune hepatitis
• Bearn-Kunkel syndrome
• Berardinelli lipodystrophy syndrome
• Bile duct stricture
• Biliary atresia
• Budd-Chiari Syndrome
• Carbohydrate deficient glycoprotein syndrome type 1a
• Cardiac cirrhosis
• Caroli disease
• Cerebrohepatorenal syndrome
• Ceroid storage disease
• Cholestasis-oedema syndrome, Norwegian type
• Cholesterol ester storage disease
• Congenital hepatic fibrosis
• Constrictive pericarditis
• Cor Pulmonale
• Cruveilhier-Baumgarten syndrome
• Cystic fibrosis
• Erythropoietic protoporphyria
• Ethanol
• Fanconi disease
• Fasciola hepatica
• Fructose-1-phosphate aldolase deficiency
• 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
• Reynolds syndrome
• Right sided cardiac failure
• Sarcoidosis
• Schistosomiasis
• Sickle Cell Disease
• Steatohepatitis
• Thalassemia
• Tricho-hepato-enteric syndrome
• Tricuspid insufficiency
• Tyrosinaemia type 1
• Visceral leishmaniasis
• Wilson disease