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Cirrhosis of the Liver

Basics

  • Chronic inflammatory disease with hepatocellular dysfunction, fibrosis, necrosis, vascular remodeling.
  • May progress to liver failure and hepatocellular carcinoma (HCC).

Epidemiology

  • Diagnosis usually between 40-60 years; males > females.
  • 12th leading cause of death in the US.
  • Incidence: β‰₯30,000 new cases/year.
  • Prevalence: 0.3% diagnosed with cirrhosis (~630,000 Americans); 2% with chronic liver disease.
  • Higher prevalence in non-Hispanic blacks, Mexican Americans, and low socioeconomic groups.

Etiology and Pathophysiology

  • Causes: HCV (26%), alcohol abuse (21%), NASH (~10%, increasing), HBV + HDV (15%), others (~25%) including hemochromatosis, autoimmune hepatitis, PBC, secondary biliary cirrhosis, biliary atresia, Wilson disease, Ξ±1-antitrypsin deficiency, granulomatous diseases, drug-induced liver disease, venous outflow obstruction (Budd-Chiari).
  • Hepatocellular injury β†’ regenerative nodules + fibrosis + angiogenesis β†’ distorted blood flow β†’ portal hypertension.
  • Genetics: hereditary hemochromatosis, Wilson disease, Ξ±1-antitrypsin deficiency.

Risk Factors

  • Alcohol abuse, intravenous drug use, obesity.

General Prevention

  • Mitigate risk factors: screen and treat hepatitis C, reduce alcohol intake.
  • Weight loss in overweight/obese patients.

Commonly Associated Conditions

  • HCV, alcohol/drug abuse, diabetes, depression, obesity.

Diagnosis

History

  • Risk factors: alcohol use, viral hepatitis, family history of liver disease or liver cancer.
  • Symptoms: fatigue, malaise, anorexia, weight loss or gain (ascites/edema), right upper quadrant pain, tea-colored urine, clay-colored stools, pruritus, bruising, bleeding, menstrual irregularities, diminished libido, erectile dysfunction.

Physical Exam

  • May be normal early; later signs of portal hypertension and hyperestrinism.
  • Skin: spider angiomas, palmar erythema, jaundice, scleral icterus, ecchymoses, caput medusae, hyperpigmentation, decreased body hair, telangiectasias.
  • Abdominal: hepatomegaly, splenomegaly, ascites (fluid wave, shifting dullness).
  • Other: gynecomastia, Dupuytren contractures, pretibial edema, clubbing (in hepatopulmonary syndrome).
  • Neurologic: asterixis, altered mental status, fetor hepaticus, muscle wasting.

Differential Diagnosis

  • Steatohepatitis, portal vein thrombosis, lymphoma, metastatic liver cancer, cardiac cirrhosis, acute alcoholic hepatitis.

Diagnostic Tests & Interpretation

  • FIB-4 index: age, AST, ALT, platelet count; values >3.25 suggest cirrhosis.
  • Labs: AST > ALT (often mild elevation), elevated ALP, GGT, bilirubin; anemia due to hemolysis or splenomegaly; thrombocytopenia (<110 x10^9/L); hypoalbuminemia, prolonged PT/INR.
  • Electrolyte abnormalities with progression: hyperammonemia, hyperkalemia, hyponatremia.
  • Hepatorenal syndrome (HRS): creatinine >1.5 mg/dL, urine sodium <10 mEq/L, low urine output.
  • Imaging: ultrasound for parenchyma, veins, hepatocellular carcinoma (HCC) screening; elastography for fibrosis.
  • Additional tests: hepatitis serologies, autoimmune markers, iron studies, ceruloplasmin for Wilson disease, Ξ±-fetoprotein for HCC.
  • Liver biopsy if noninvasive tests inconclusive.
  • Endoscopy for varices and portal hypertensive gastropathy.

Treatment

General Measures

  • Abstain from alcohol, hepatotoxic drugs/herbs.
  • Vaccinations: pneumococcal, hepatitis A and B, influenza.
  • Weight loss, exercise, control lipids and glucose.
  • Assess for and manage complications: ascites, spontaneous bacterial peritonitis (SBP), hepatic encephalopathy (HE), coagulopathy, variceal hemorrhage.

Medication

  • Treat underlying cause (HCV, HBV, NASH, alcoholic hepatitis, hemochromatosis, PBC, Wilson disease, autoimmune hepatitis).
  • Esophageal varices prophylaxis: nonselective beta-blockers (propranolol, carvedilol, nadolol).
  • Ascites: low sodium diet (<2 g/day), spironolactone Β± furosemide, water restriction if hyponatremia, SBP prophylaxis in select patients.
  • HE: lactulose titrated for 2-3 soft stools daily; rifaximin added if needed.
  • Renal insufficiency: stop nephrotoxic drugs; volume management.
  • Antibiotics prophylaxis for invasive procedures and GI bleeding.

Second Line

  • HBV second-line agents: adefovir; avoid lamivudine first-line due to resistance.

Issues for Referral

  • Liver transplant evaluation at ascites, variceal bleeding, HE, jaundice, liver lesion, or hepatic dysfunction (Child-Pugh >7, MELD >10).

Surgery/Other Procedures

  • Variceal ligation (4-6 sessions).
  • Octreotide for acute variceal bleeding.
  • TIPS as salvage or second-line.
  • Therapeutic paracentesis for tense ascites.
  • Liver transplantation for fulminant hepatic failure or advanced HCC.
  • Radiofrequency ablation or resection for small HCC.

Complementary & Alternative Medicine

  • Zinc sulfate for dysgeusia, appetite, and HE adjunct.
  • Milk thistle may lower transaminases.
  • Vitamin E cautiously in NASH.

Admission, Inpatient, and Nursing Considerations

  • Admit for major GI bleeding, AMS, sepsis/infection, rapid decompensation, renal failure.

Ongoing Care

  • Monitor liver enzymes, sodium, creatinine, platelets, PT every 6-12 months.
  • MELD score calculation.
  • Ultrasound and Ξ±-fetoprotein screening for HCC every 6-12 months.
  • MRI for suspicious lesions.
  • EGD screening for varices at diagnosis and regularly thereafter.

Follow-Up Recommendations

  • Regular physical conditioning.
  • Monitor fatigue, weakness, appetite, pruritus, AMS, jaundice, abdominal pain.

Diet

  • Protein 1.2-1.5 g/kg/day, high fiber, multivitamins (without iron).
  • Sodium <2 g/day; fluid restriction if ascites/edema.

Patient Education

  • Emergency signs: hematemesis, AMS.
  • Abstain from alcohol, drugs, smoking.
  • Update vaccinations.
  • HCV transmission precautions.

Prognosis

  • 5-20 years asymptomatic.
  • Death typically within 5 years after complications.
  • 5% annual HCC development risk.
  • 50% develop ascites over 10 years; 50% 5-year survival after ascites.
  • Acute variceal bleeding mortality ~30%.
  • Transplant survival 85% at 1 year; annual mortality ~5%.

Complications

  • Ascites, edema, infections, HE, GI bleeding, varices, portal gastropathy, hepatorenal syndrome, hepatopulmonary syndrome, HCC, liver failure.

Clinical Pearls:

  • 80% of chronic liver disease is preventable, mainly by addressing alcohol and viral hepatitis.
  • Ultrasound screening for HCC every 6-12 months after cirrhosis diagnosis.
  • Always rule out spontaneous bacterial peritonitis in new ascites.