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Cirrhosis, Emergency Medicine

Basics

Description

- Drugs: - Acetaminophen - Methotrexate - Amiodarone - Methyldopa

- Hepatic congestion: - Right-sided heart failure - Pericarditis - Budd-Chiari syndrome (hepatic venous outflow obstruction)

- Infiltrative: - Sarcoidosis - Amyloidosis - Nonalcoholic steatohepatitis (NASH) - Hepatocellular carcinoma, diffusely infiltrating

- Infections: - Brucellosis - Echinococcosis - Tertiary syphilis - Schistosomiasis

- Hepatopulmonary syndrome: - Intrapulmonary vascular dilation and hypoxia - Results in increased alveolar-arterial gradient

- CBC: - Anemia - Macrocytosis - Leukopenia and neutropenia - Thrombocytopenia

- Increased liver enzymes: - Aspartate alanine aminotransferase (AST, SGOT), alanine aminotransferase (ALT, SGPT)-reflect injury - Ratio of AST:ALT ≥2 in alcoholic liver disease - Alkaline phosphatase and 5"пїЅ-nucleotidase reflect cholestasis. - Оі-Glutamyltranspeptidase (GGT) - May be normal in inactive cirrhosis

- Search for cause: - Hepatitis B surface antigen - Hepatitis C antibody - Antinuclear antibody (ANA) and antismooth muscle antibody (autoimmune hepatitis) - Antimitochondrial antibody (PBC) - Serum iron, transferrin saturation, and ferritin (hemochromatosis) - Ceruloplasmin (Wilson disease) - О±1-Antitrypsin deficiency - Serum immune electrophoresis (high IgM in PBC) - Cholesterol (chronic cholestasis) - О±-Fetoprotein (hepatocellular cancer)

- Encephalopathy: - Metabolic - Toxic - Intracranial process

- For suspected variceal bleed: - IV proton pump inhibitors - IV octreotide-splanchnic vasoconstrictor - Reverse coagulopathy: - Fresh-frozen plasma 1 IU/hr until bleeding is controlled - Desmopressin (DDAVP)-improves bleeding time and prolonged PTT

- Initiate broad-spectrum antibiotics in suspected sepsis or SBP: - Cefotaxime - Ticarcillin-clavulanate - Piperacillin-tazobactam - Ampicillin-sulbactam

- β-Blocker (propranolol) for esophageal varices: - Titrated to pulse rate of 60 or 25% reduction of resting pulse - With or without isosorbide dinitrate - Decreases rebleeding rate - May delay or prevent occurrence of 1st bleed

  • Progressive process of inflammation, cellular injury and necrosis, diffuse fibrosis, and formation of regenerative nodules
  • Loss of lobular and vascular architecture
  • Irreversible in advanced stages
  • Intrahepatic portal hypertension owing to increased resistance at the sinusoid, compression of the central veins, and anastomosis between the arterial and portal systems
  • 10th leading cause of death in US

Etiology

  • Chronic alcohol abuse (most common cause in US)
  • Chronic viral hepatitis, B or C (2nd most common cause in US)
  • Autoimmune hepatitis
  • Biliary cirrhosis, primary (PBC) or secondary (sclerosing cholangitis)
  • Metabolic:Hereditary hemochromatosisWilson diseasePorphyria
  • Drugs:AcetaminophenMethotrexateAmiodaroneMethyldopa
  • Hepatic congestion:Right-sided heart failurePericarditisBudd-Chiari syndrome (hepatic venous outflow obstruction)
  • Infiltrative:SarcoidosisAmyloidosisNonalcoholic steatohepatitis (NASH)Hepatocellular carcinoma, diffusely infiltrating
  • Infections:BrucellosisEchinococcosisTertiary syphilisSchistosomiasis
  • Congenital
  • Arteriohepatic dysplasia, biliary atresia, cystic fibrosis, О±1-antitrypsin deficiency
  • Metabolic
  • Fructosemia, tyrosinemia, galactosemia, glycogen storage diseases
  • Infectious
  • Congenital hepatitis B

Diagnosis

Signs and Symptoms

  • May be silent
  • Insidious onset with nonspecific findings:MalaiseFatigueAnorexiaNausea and vomitingWeight lossPruritusHyperpigmentation
  • Jaundice
  • Abdominal collateral circulation including caput medusae
  • Hepatomegaly
  • Splenomegaly
  • Abdominal discomfort or tenderness
  • Fever
  • Fetor hepaticus
  • Asterixis
  • Hypotension
  • Cruveilhier-Baumgarten murmur
  • Renal insufficiency
  • Spider telangiectasias
  • Palmar erythema
  • Dupuytren contractures
  • Parotid and lacrimal gland enlargement
  • Terry nails
  • Muehrcke lines
  • Clubbing
  • Feminization:Testicular atrophyImpotenceLoss of libidoGynecomastia
  • Amenorrhea
  • Complications:When complications develop, patient is considered to have decompensated disease.AscitesSpontaneous bacterial peritonitis (SBP)Hepatic encephalopathy (HE)-may be precipitated by:GI bleedInfectionsIncreased dietary proteinHypokalemiaSedativesConstipationAzotemiaAlkalosisVariceal hemorrhage:1/3 of patients with variceal bleed.Each bleeding episode carries a 33% mortality rate.Hepatic venous pressure gradient >12 mm Hg increases risk of bleed.Portal hypertensive gastropathy or peptic ulcer diseaseHepatorenal failure:Caused by decreased renal perfusion during severe decompensated cirrhosisMay be iatrogenic: Secondary to diuretics, NSAIDs, IV contrast, aminoglycosides, large-volume paracentesisHigh mortality rateHepatopulmonary syndrome:Intrapulmonary vascular dilation and hypoxiaResults in increased alveolar-arterial gradient

Essential Workup

Detailed history and physical exam to search for clues to liver disease пїЅ

Diagnosis Tests & Interpretation

Lab

  • CBC:AnemiaMacrocytosisLeukopenia and neutropeniaThrombocytopenia
  • Impaired liver function:High bilirubinLow albuminHigh globulinsProlonged PTVarying degrees of DICHypoglycemia
  • Increased liver enzymes:Aspartate alanine aminotransferase (AST, SGOT), alanine aminotransferase (ALT, SGPT)-reflect injuryRatio of AST:ALT ≥2 in alcoholic liver diseaseAlkaline phosphatase and 5"пїЅ-nucleotidase reflect cholestasis.Оі-Glutamyltranspeptidase (GGT)May be normal in inactive cirrhosis
  • Electrolytes, BUN, and creatinine
  • Hyponatremia:Renal dysfunction and hepatorenal syndrome
  • Arterial blood gases or pulse oximeter for:Suspected pneumoniaCHFHepatopulmonary syndrome
  • Search for cause:Hepatitis B surface antigenHepatitis C antibodyAntinuclear antibody (ANA) and antismooth muscle antibody (autoimmune hepatitis)Antimitochondrial antibody (PBC)Serum iron, transferrin saturation, and ferritin (hemochromatosis)Ceruloplasmin (Wilson disease)О±1-Antitrypsin deficiencySerum immune electrophoresis (high IgM in PBC)Cholesterol (chronic cholestasis)О±-Fetoprotein (hepatocellular cancer)

Imaging

  • US for liver architecture, biliary obstruction, ascites, portal vein thrombosis, splenomegaly
  • CT scan to explore abnormal finding on ultrasound
  • CXR for pleural effusion, cardiomegaly, and CHF

Diagnostic Procedures/Surgery

  • Esophagogastroduodenoscopy (EGD) indicated for upper GI bleeding or variceal surveillance
  • Variceal ligation or endoscopic sclerotherapy
  • Paracentesis for significant ascites or SBP

Differential Diagnosis

  • Ascites:Increased right heart pressureHepatic vein thrombosisPeritoneal malignancy/infectionPancreatic diseaseThyroid diseaseLymphatic obstruction
  • Upper GI bleeding:Peptic ulcer diseaseGastritis
  • Encephalopathy:MetabolicToxicIntracranial process

Treatment

Pre-Hospital

  • Naloxone, dextrose (or Accu-Chekk), and thiamine for altered mental status
  • Reverse hypotension with IV fluids to prevent acute ischemic hepatic injury.

Initial Stabilization/Therapy

Treat complications such as GI bleeding or HE. пїЅ

Ed Treatment/Procedures

  • For suspected variceal bleed:IV proton pump inhibitorsIV octreotide-splanchnic vasoconstrictorReverse coagulopathy:Fresh-frozen plasma 1 IU/hr until bleeding is controlledDesmopressin (DDAVP)-improves bleeding time and prolonged PTTBalloon tamponade with Sengstaken-Blakemore tube or a variant for variceal compression (rarely used anymore, prophylactic intubation recommended)Emergent endoscopic sclerotherapy
  • Initiate broad-spectrum antibiotics in suspected sepsis or SBP:CefotaximeTicarcillin-clavulanatePiperacillin-tazobactamAmpicillin-sulbactam
  • Treat complicating conditions such as ascites, HE, SBP.
  • Treat pruritus with:Diphenhydramine 25-50 mg IM/IV q4hCholestyramine, ursodeoxycholic acid, or rifampinNaloxone infusion 0.2 Ојg/kg/min for temporary relief for extreme cases
  • β-Blocker (propranolol) for esophageal varices:Titrated to pulse rate of 60 or 25% reduction of resting pulseWith or without isosorbide dinitrateDecreases rebleeding rateMay delay or prevent occurrence of 1st bleed
  • Relieve biliary obstruction (e.g., stricture) by endoscopic, radiologic, or surgical means.
  • Provide nutritious diet, high in calories and adequate in protein (1 g/kg), unless there is complicating HE
  • Consult transplantation coordinator whenever postliver transplantation patient presents to the ED with liver dysfunction, suspected sepsis, or possible treatment-related complication.

Special Therapy

  • Hemochromatosis: Phlebotomy or deferoxamine (iron-chelating agent)
  • Autoimmune hepatitis: Prednisone with or without azathioprine
  • Chronic hepatitis B or C: О±-Interferon (avoid in decompensated cirrhosis)
  • PBC: Ursodeoxycholic acid
  • Wilson disease: Penicillamine
  • The only cure for most advanced cirrhosis is liver transplantation.

Medication

  • Azathioprine: 1-2 mg/kg PO daily
  • Cefotaxime: 1-2 g q6-8h (peds: 50-180 mg/kg/d q6h) IV
  • Cholestyramine: 4 g PO 1-6 times per day
  • Desmopressin (DDAVP): 0.3 Ојg/kg in 50 mL saline infused over 15-30 min
  • Dextrose: D50W 1 amp (50 mL or 25 g; peds: D25W 2-4 mL/kg) IV
  • Naloxone: 0.2-2 mg (peds: 0.1 mg/kg) IV or IM initial dose
  • Lactulose: 15-30 mL TID-goal is 2-3 stools per day
  • Octreotide: 25-50 Ојg IV bolus followed by 50 Ојg/hr IV infusion
  • Piperacillin-tazobactam: 3.375 g IV q6h (peds: 100-400 mg/kg/d div. q6-8h; renal dosing required)
  • Prednisone: 40 mg (peds: 1-2 mg/kg) PO daily
  • Propranolol: 40 (initial) to 240 mg (peds: 1-5 mg/kg/d) PO TID
  • Rifampin: 600 mg (peds: 10-20 mg/kg) PO daily
  • Thiamine: 100 mg (peds: 50 mg) IV or IM
  • Ursodeoxycholic acid: 8-10 mg/kg/d TID

Follow-Up

Disposition

Admission Criteria

  • Acute decompensation or complicating conditions
  • 1st presentation with clinically evident cirrhosis, unless close outpatient workup is possible
  • Advanced grades HE, sepsis, active GI bleed, and hepatorenal and hepatopulmonary syndromes require ICU.
  • Advanced stages of hepatocellular carcinoma

Discharge Criteria

Most patients with compensated cirrhosis can be treated as outpatients. пїЅ

Followup Recommendations

GI for all new cases пїЅ

Pearls and Pitfalls

  • Prognosis is highly variable.
  • Patients present with a wide variety of signs and symptoms related to end-stage liver disease.
  • New cases need full workup and GI consultation for management.
  • Any complication puts patient in decompensated state.
  • SBP symptoms are frequently vague:Must have a high suspicion and low threshold for paracentesis when considering SBP

Additional Reading

  • Feldman пїЅM. Sleisenger and Fordtrans Gastrointestinal and Liver Disease. 9th ed. Philadelphia, PA: WB Saunders; 2010.
  • Goldberg пїЅE. Diagnostic Approach to the Patient with Cirrhosis. Wellesley, MA: UpToDate; 2012.
  • Longo пїЅD, Fauci пїЅA, et al. Harrison's Principles of Internal Medicine. 18th ed. New York, NY: McGraw-Hill; 2011.
  • Runyon пїЅBA. Management of adult patients with ascites due to cirrhosis. Hepatology. 2009;49(6):2087-2107.

See Also (Topic, Algorithm, Electronic Media Element)

  • Ascites
  • Hepatic Encephalopathy
  • Hepatitis
  • Spontaneous Bacterial Peritonitis
  • Varices

Codes

ICD9

  • 571.2 Alcoholic cirrhosis of liver
  • 571.5 Cirrhosis of liver without mention of alcohol
  • 571.6 Biliary cirrhosis

ICD10

  • K70.30 Alcoholic cirrhosis of liver without ascites
  • K74.5 Biliary cirrhosis, unspecified
  • K74.60 Unspecified cirrhosis of liver
  • K74.69 Other cirrhosis of liver
  • K70.31 Alcoholic cirrhosis of liver with ascites
  • K70.3 Alcoholic cirrhosis of liver
  • K74.3 Primary biliary cirrhosis
  • K74.4 Secondary biliary cirrhosis
  • K74.6 Other and unspecified cirrhosis of liver

SNOMED

  • 19943007 Cirrhosis of liver (disorder)
  • 420054005 Alcoholic cirrhosis (disorder)
  • 266468003 Cirrhosis - non-alcoholic (disorder)
  • 1761006 Biliary cirrhosis (disorder)
  • 31712002 Primary biliary cirrhosis (disorder)