Cirrhosis, Emergency Medicine

Basics

Description

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 hemochromatosis
- Wilson disease
- Porphyria
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

Congenital
Arteriohepatic dysplasia, biliary atresia, cystic fibrosis, α1-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:
- Malaise
- Fatigue
- Anorexia
- Nausea and vomiting
- Weight loss
- Pruritus
- Hyperpigmentation
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 atrophy
- Impotence
- Loss of libido
- Gynecomastia
Amenorrhea
Complications:
- When complications develop, patient is considered to have decompensated disease.
- Ascites
- Spontaneous bacterial peritonitis (SBP)
- Hepatic encephalopathy (HE)-may be precipitated by:
  - GI bleed
  - Infections
  - Increased dietary protein
  - Hypokalemia
  - Sedatives
  - Constipation
  - Azotemia
  - Alkalosis
- Variceal 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 disease
- Hepatorenal failure:
  - Caused by decreased renal perfusion during severe decompensated cirrhosis
  - May be iatrogenic: Secondary to diuretics, NSAIDs, IV contrast, aminoglycosides, large-volume paracentesis
  - High mortality rate
- Hepatopulmonary syndrome:
  - Intrapulmonary vascular dilation and hypoxia
  - Results in increased alveolar-arterial gradient

Essential Workup

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

Diagnosis Tests & Interpretation

Lab

CBC:
- Anemia
- Macrocytosis
- Leukopenia and neutropenia
- Thrombocytopenia
Impaired liver function:
- High bilirubin
- Low albumin
- High globulins
- Prolonged PT
- Varying degrees of DIC
- Hypoglycemia
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"�-nucleotidase reflect cholestasis.
- γ-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 pneumonia
- CHF
- Hepatopulmonary syndrome
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)
- α1-Antitrypsin deficiency
- Serum immune electrophoresis (high IgM in PBC)
- Cholesterol (chronic cholestasis)
- α-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 pressure
- Hepatic vein thrombosis
- Peritoneal malignancy/infection
- Pancreatic disease
- Thyroid disease
- Lymphatic obstruction
Upper GI bleeding:
- Peptic ulcer disease
- Gastritis
Encephalopathy:
- Metabolic
- Toxic
- Intracranial 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. �

Ed Treatment/Procedures

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
- Balloon 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:
- Cefotaxime
- Ticarcillin-clavulanate
- Piperacillin-tazobactam
- Ampicillin-sulbactam
Treat complicating conditions such as ascites, HE, SBP.
Treat pruritus with:
- Diphenhydramine 25-50 mg IM/IV q4h
- Cholestyramine, ursodeoxycholic acid, or rifampin
- Naloxone infusion 0.2 μg/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 pulse
- With or without isosorbide dinitrate
- Decreases rebleeding rate
- May 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: α-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 μg/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 μg IV bolus followed by 50 μg/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. �

Followup Recommendations

GI for all new cases �

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

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)

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