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.