Cirrhosis Disease

BASICS

DESCRIPTION

Histologically, cirrhosis is defined by the presence of fibrosis with regenerative nodules. Clinically, cirrhosis presents with evidence of portal hypertension, i.e. ascites, variceal bleeding, hepatic encephalopathy.

System(s) affected: Gastrointestinal, Cardiovascular, Endocrine/Metabolic
Genetics: For hereditary hemochromatosis HFe gene mutation C282Y; for Wilson's disease mutation on chromosome 13
Incidence/Prevalence in USA: Accounts for over 30,000 deaths per year
Predominant age: Etiology dependent
Predominant sex: Etiology dependent

SIGNS AND SYMPTOMS

The onset of the disease is often insidious with:
- Fatigue
- Anorexia
- Nausea
- Abdominal discomfort and distention
- Weakness and malaise
Signs and symptoms that are related to cirrhosis are those of complications:
- Hematemesis
- Encephalopathy
- Jaundice
- Hepatomegaly
- Splenomegaly
- Abdominal collateral circulation
- Ascites
- Gynecomastia
- Testicular atrophy
- Asterixis (liver flap)
- Palmar erythema
- Spider angiomas

CAUSES

Alcoholic cirrhosis
Chronic viral hepatitis, B (with/without D), C
Wilson's disease
Hemochromatosis
Alpha 1-antitrypsin deficiency
Cystic fibrosis
Autoimmune chronic hepatitis with cirrhosis
Primary biliary cirrhosis
Secondary biliary cirrhosis
Primary sclerosing cholangitis
Cardiac cirrhosis
Drug induced (other than alcohol)
Nonalcoholic steatohepatitis (NASH)
Inherited causes that may be present in infancy and childhood:
- Glycogen storage disease
- Galactosemia
- Fructose intolerance
- Tyrosinemia
- Acid cholesterol ester hydrolase deficiency

RISK FACTORS

Alcohol use
Hepatotoxic drugs
Excessive iron ingestion

DIAGNOSIS

LABORATORY

Recognition of liver injury - elevated AST, elevated ALT; elevated alkaline phosphatase. Note: All liver injury tests may be normal.
Functional impairment of the liver - elevated bilirubin, decreased albumin, elevated globulin, prolonged prothrombin time
Etiologic screen for liver disease
- Ceruloplasmin (Wilson's disease)
- Iron, iron binding capacity, ferritin (hemochromatosis)
- Alpha fetoprotein (hepatocellular cancer)
- HBsAg (hepatitis B)
- Anti-HCV (hepatitis C)
- HCV RNA by PCR to confirm activity
- ANA (autoimmune hepatitis)
- Anti-smooth muscle antibody (autoimmune hepatitis)
- Anti-mitochondrial antibody (AMA) (primary biliary cirrhosis)
- Alpha 1-antitrypsin (deficiency)
- Serum protein electrophoresis (SPEP) - increased IgG with any liver disease; increased IgM with primary biliary cirrhosis (PBC)

Drugs that may alter lab results: N/A
Disorders that may alter lab results: N/A

PATHOLOGICAL FINDINGS

Fibrosis and regenerative nodules; specific findings/patterns may indicate etiology
Quantitative liver chemistry for iron, copper
Special stains for iron, copper, bilirubin, collagen, alpha 1-antitrypsin, hepatitis B

SPECIAL TESTS

Laparoscopic liver biopsy to reduce sampling error
Cholangiography by endoscopy and MRI to rule out common duct obstruction and recognize sclerosing cholangitis
Doppler ultrasound to indicate direction of flow in and patency of the portal and hepatic veins
Visceral angiography to determine vascular anatomy, patency and collaterals
Esophagogastroduodenoscopy to assess varices

IMAGING

Ultrasound good for detecting bile duct dilatation and space occupying lesions. Cannot make diagnosis of cirrhosis based on ultrasound alone.
CT, if ultrasound technically inadequate

DIAGNOSTIC PROCEDURES

A liver biopsy establishes the diagnosis of cirrhosis
Patterns of injury as well as special stains may identify a precise etiology such as alcoholic liver disease, hemochromatosis, alpha 1-antitrypsin deficiency, hepatitis B, primary biliary cirrhosis
Level of activity determined

TREATMENT

APPROPRIATE HEALTH CARE

Outpatient except for complicating emergencies:
- GI bleeding
- Hepatic encephalopathy
- Spontaneous bacterial peritonitis
- Unexplained decompensation
- Renal failure

GENERAL MEASURES

Treatment designed to remove or alleviate underlying cause of cirrhosis, prevent further liver damage and prevent complications
Phlebotomy for hemochromatosis
Therapies involve drug treatment, dietary restrictions, rest, other supportive measures. Adequate protein intake for liver regeneration.

SURGICAL MEASURES

Interventional
- Possible procedures for portal hypertension include - splenorenal or portacaval anastomosis, transjugular intrahepatic portal-systemic shunt
- Transplantation - in suitable candidate (substance free, motivated and adherent). Evaluate prior to major decompensation.

ACTIVITY

Maintain as active as possible. With peripheral edema, leg elevation necessary.

DIET

Adequate protein (1 gm/kg) and generous calories to help regenerate the liver
In the presence of hepatic encephalopathy protein restriction is necessary
In the presence of ascites salt restriction is necessary (2 gm or less/day)
In the presence of hyponatremia (Na < 130 mEq [< 130 mmol]) fluid restriction is necessary (< 1 L)
No alcohol

PATIENT EDUCATION

Pamphlets are available through: American Liver Foundation, (800)223-0179
Additional material: National Digestive Diseases Information Clearinghouse, Box NDDIC, Bethesda, MD 20892, (301)468-6344

FOLLOW UP

PREVENTION/AVOIDANCE

Limit use of alcohol and other liver toxins
No sharing of syringes
Safer sex
Screening of family members when a genetic disease is recognized
Influenza and pneumococcal vaccines for cirrhosis patients exposed to crowds
Hepatitis A and B vaccines
Liver test surveillance while on hepatotoxic drugs (e.g., INH)

POSSIBLE COMPLICATIONS

Infection is common
Cor pulmonale, secondary polycythemia, bullous lung disease, acute or chronic respiratory failure, pulmonary hypertension, malnutrition, pneumothorax

EXPECTED COURSE AND PROGNOSIS

A function of ongoing hepatic injury as well as residual hepatic reserve. If a treatable cause is identified and intervention results in cessation of liver destruction, then the prognosis may be good.

MISCELLANEOUS

ASSOCIATED CONDITIONS

Hepatitis
Diseases and defects of the bile ducts
Cystic fibrosis
Heart failure
Hepatocellular cancer

AGE-RELATED FACTORS

Pediatric: N/A
Geriatric: Cirrhosis is one of the leading causes of death for people over age 65
Others: N/A

PREGNANCY

Cirrhosis may decompensate during pregnancy. Higher rates of spontaneous abortion, premature birth and perinatal death.

OTHER NOTES

N/A

ABBREVIATIONS

PBC = Primary biliary cirrhosis
UGI = upper gastrointestinal
ANA = antinuclear antibody

Clinical Investigations

ROLE OF HOMOEOPATHY

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