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Basic Information ⬇

AUTHOR: David J. Lucier Jr., MD, MBA, MPH

Definition

Cirrhosis is defined histologically as the presence of irreversible fibrosis in the liver. It can be classified as micronodular, macronodular, or mixed; however, each form may be seen in the same patient at different stages of the disease, and this classification system has little utility in determining the underlying etiology of cirrhosis. Cirrhosis manifests clinically with portal hypertension, ascites and peripheral edema, hepatic encephalopathy, and variceal bleeding.

ICD-10CM CODES
K70.30Alcoholic cirrhosis of liver without ascites
K70.31Alcoholic cirrhosis of liver with ascites
K71.7Toxic liver disease with fibrosis and cirrhosis of liver
K74.3Primary biliary cirrhosis
K74.4Secondary biliary cirrhosis
K74.5Biliary cirrhosis, unspecified
K74.60Unspecified cirrhosis of liver
K74.69Other cirrhosis of liver
P78.81Congenital cirrhosis (of liver)
Epidemiology & Demographics

  • Cirrhosis was the fourteenth-leading cause of death in the U.S. in 2015 and the thirteenth-leading cause of death globally. In the U.S., cirrhosis-related deaths increased 3.4% annually between 2009 and 2016.
  • Alcohol abuse, hepatitis C, and nonalcoholic steatohepatitis are the major causes of cirrhosis in the U.S.
  • Economic burden of cirrhosis in the U.S. exceeds $2 billion in direct costs and over $10 billion in indirect costs.
Physical Findings & Clinical Presentation
General

Fever (spontaneous bacterial peritonitis)

Skin

Jaundice, palmar erythema, spider angiomata, ecchymosis (thrombocytopenia or coagulation factor deficiency), increased pigmentation (hemochromatosis), xanthomas (primary biliary cirrhosis), and diffuse pruritus. Cutaneous lesions often accompany cirrhosis and can be found in >40% of people with chronic alcoholism

Eyes

Kayser-Fleischer rings (corneal copper deposition seen in Wilson disease; best diagnosed with slit lamp examination), scleral icterus

Breath

Fetor hepaticus (breath has a sweet musty odor found in cirrhosis with hepatic failure)

Chest

Possible gynecomastia in men

Abdomen

Tender or nontender hepatomegaly (congestive hepatomegaly), small, nodular liver (cirrhosis), palpable, nontender gallbladder (neoplastic extrahepatic biliary obstruction), palpable spleen (portal hypertension), dilated superficial periumbilical vein (caput medusae), venous hum auscultated over periumbilical veins (portal hypertension), ascites (portal hypertension, hypoalbuminemia), diffuse abdominal tenderness (spontaneous bacterial peritonitis)

Rectal Examination

Hemorrhoids (portal hypertension), guaiac-positive stools (alcoholic gastritis, bleeding esophageal varices, peptic ulcer disease, bleeding hemorrhoids, portal gastropathy)

Genitalia

Testicular atrophy in males (chronic liver disease, hemochromatosis)

Extremities

Pedal edema (hypoalbuminemia, anasarca), arthropathy (hemochromatosis), Dupuytren contractures

Neurologic

Asterixis (hepatic encephalopathy), choreoathetosis, dysarthria (Wilson disease)

Etiology

  • Chronic hepatitis B virus (HBV) and hepatitis C virus (HCV) infection
  • Alcoholism
  • Nonalcoholic steatohepatitis
  • Primary biliary cholangitis
  • Secondary biliary cirrhosis, obstruction of the common bile duct (stone, stricture, pancreatitis, neoplasm, sclerosing cholangitis)
  • Autoimmune hepatitis
  • Drugs with chronic hepatitis (e.g., acetaminophen, isoniazid, methotrexate, methyldopa)
  • Chronic hepatic congestion (e.g., congestive heart failure [CHF], constrictive pericarditis, tricuspid insufficiency, thrombosis of the hepatic vein, obstruction of the vena cava)
  • Hemochromatosis
  • Wilson disease
  • Alpha-1-antitrypsin deficiency
  • Infiltrative diseases (amyloidosis, glycogen storage diseases, nonhepatocellular malignancies)
  • Nutritional: Jejunoileal bypass
  • Others: Parasitic infections (schistosomiasis), idiopathic portal hypertension, congenital hepatic fibrosis, systemic mastocytosis

Diagnosis ⬆ ⬇

Workup

In addition to an assessment of liver function, the evaluation of patients with cirrhosis should also include an assessment of renal and circulatory function. Diagnostic workup is aimed primarily at identifying the most likely cause of cirrhosis. The history is extremely important:

  • Alcohol abuse: Alcoholic liver disease
  • History of hepatitis B or hepatitis C
  • Obesity, type 2 diabetes mellitus, hyperlipidemia (nonalcoholic steatohepatitis)
  • History of inflammatory bowel disease (IBD; primary sclerosing cholangitis)
  • History of pruritus, hyperlipoproteinemia, and xanthomas in a middle-aged or elderly woman (primary biliary cholangitis)
  • Impotence, diabetes mellitus, hyperpigmentation, arthritis (hemochromatosis)
  • Neurologic disturbances (Wilson disease, hepatolenticular degeneration)
  • Family history of “liver disease” (hemochromatosis [positive family history in 25% of patients], alpha-1-antitrypsin deficiency)
  • History of recurrent episodes of right upper quadrant pain (biliary tract disease)
  • History of blood transfusions, IV drug abuse (hepatitis C)
  • History of repetitive hepatotoxic drug exposure
  • Coexistence of other diseases with immune or autoimmune features (immune thrombocytopenic purpura, myasthenia gravis, thyroiditis, autoimmune hepatitis)
  • Biopsy is the gold standard to diagnosis cirrhosis and is helpful when multiple etiologies are possible that might change management (autoimmune hepatitis, small duct primary sclerosing cholangitis, antimitochondrial antibody-negative primary biliary cholangitis, and infiltrative diseases such as lymphoma, amyloidosis, and granulomatous hepatitis). However, it is generally unnecessary if the clinical picture is highly suggestive of cirrhosis and management would not change. Biopsy can be useful in actively drinking patients with cirrhosis to distinguish between decompensated cirrhosis and cirrhosis with alcoholic hepatitis
Laboratory Tests

  • Decreased hemoglobin and hematocrit, elevated mean corpuscular volume (Fig. E1). Increased blood urea nitrogen (BUN) and creatinine (the BUN may also be “normal” or low if the patient has severely diminished liver function).
  • Decreased sodium (dilutional hyponatremia), and decreased potassium (as a result of secondary aldosteronism or urinary losses). Evaluation of renal function should also include measurement of urinary sodium and urinary protein from 24-hr urine collection.
  • Decreased glucose in a patient with liver disease indicates severe liver damage.
  • Other laboratory abnormalities:
    1. Alcoholic hepatitis and cirrhosis: Possible mild elevation of alanine aminotransferase (ALT) and aspartate aminotransferase (AST), usually <500 IU; AST >ALT (ratio >2:3).
    2. Extrahepatic obstruction: Possible moderate elevations of ALT and AST to levels <500 IU.
    3. Viral, toxic, or ischemic hepatitis: Extreme elevations (>500 IU) of ALT and AST.
    4. Transaminases may be normal despite significant liver disease in patients with jejunoileal bypass operations or hemochromatosis or after methotrexate administration.
    5. Alkaline phosphatase elevation can occur with extrahepatic obstruction, primary biliary cholangitis, and primary sclerosing cholangitis.
    6. Serum lactate dehydrogenase is significantly elevated in metastatic disease of the liver; lesser elevations are seen with hepatitis, cirrhosis, extrahepatic obstruction, and congestive hepatomegaly.
    7. Serum gamma-glutamyl transpeptidase is elevated in alcoholic liver disease and may also be elevated with cholestatic disease (primary biliary cholangitis, primary sclerosing cholangitis).
    8. Serum bilirubin may be elevated; urinary bilirubin can be present in hepatitis, hepatocellular jaundice, and biliary obstruction.
    9. Serum albumin: Significant liver disease results in hypoalbuminemia. Malnutrition occurs in 20% to 60% of patients with cirrhosis.
  • Prothrombin time/international normalized ratio (INR): Elevation in patients with liver disease indicates severe liver damage and poor prognosis. Table 1 summarizes hemostatic balance in liver disease.
    1. Presence of hepatitis B surface antigen implies acute or chronic hepatitis B.
    2. Presence of antimitochondrial antibody suggests primary biliary cholangitis, chronic hepatitis.
    3. Elevated serum copper, decreased serum ceruloplasmin, and elevated 24-hr urine may be diagnostic of Wilson disease.
    4. Protein immunoelectrophoresis may reveal decreased α-1 globulins (alpha-1-antitrypsin deficiency), increased IgA (alcoholic cirrhosis), increased IgM (primary biliary cirrhosis), increased IgG (chronic hepatitis, cryptogenic cirrhosis).
    5. An elevated serum ferritin and increased iron saturation are suggestive of hemochromatosis.
    6. An elevated blood ammonia suggests hepatocellular dysfunction; serial values, however, are generally not useful in monitoring patients with hepatic encephalopathy because there is poor correlation between blood ammonia level and degree of hepatic encephalopathy.
    7. Serum cholesterol is elevated in cholestatic disorders.
    8. Antinuclear antibodies (ANA) may be found in autoimmune hepatitis.
    9. Alpha fetoprotein: Levels >1000 pg/ml are highly suggestive of hepatocellular carcinoma.
    10. Hepatitis C viral testing identifies patients with chronic hepatitis C infection.
    11. Elevated level of serum globulin (especially gamma-globulins) and positive ANA test may occur with autoimmune hepatitis.
    12. End-stage liver disease is characterized by decreased levels of most procoagulant factors with the notable exceptions of factor VIII and von Willebrand factor, which are elevated. Table 2 summarizes hemostatic indices in liver disease.

TABLE 1 Hemostatic Balance in Liver Disease

Promotes ThrombosisPromotes Bleeding
Primary hemostasis
  • Increased vWF
  • Decreased ADAMTS13
  • Thrombocytopenia
  • Platelet dysfunction
Secondary hemostasis
  • Increased factor VIII
  • Decreased protein C, protein S, antithrombin
  • Factor deficiencies: II, V, VII, IX, XI
  • Vitamin K deficiency
  • Hypofibrinogenemia
  • Dysfibrinogenemia
Fibrinolysis
  • Reduced plasminogen
  • Increased PAI-1
  • Reduced α2-antiplasmin, TAFI, factor VIII
  • Increased t-PA

ADAMST13, A disintegrin and metalloproteinase with thrombospondin; PAI-1, plasminogen activator inhibitor-1; TAFI, thrombin activatable fibrinolysis inhibitor; t-PA, tissue plasminogen activator; vWF, von Willebrand factor.

From Hoffman R et al: Hematology: basic principles and practice, ed 7, Philadelphia, 2018, Elsevier.

TABLE 2 Hemostatic Indices in Liver Disease

Laboratory ChangesPTPTTTCTFibClaussPltPlatelet AggregationFVIIDDELT
ThrombocytopeniaNNNNN↓NNNN
Platelet dysfunctionNNNNNNAbnormalNNN
Vitamin K deficiencya↑↑NNNNN↓NN
Factor deficiency↑↑NNNNN↓NN
HypofibrinogenemiaN/↑N/↑↑↓↓NNNNN
DysfibrinogenemiaN/↑N/↑↑N↓NNNNN
HyperfibrinolysisN/↑N/↑N/↑N/↓N/↓NN↓↑↓
DICN/↑N/↑N/↑↓↓↓NN/↓↑↓

Clauss, Clauss fibrinogen; DD, D-dimer; DIC, disseminated intravascular coagulation; ELT, euglobulin lysis time (measure of fibrinolysis); Fib, fibrinogen; FVII, factor VII functional assay; N, normal; Plt, platelet; PT, prothrombin time; PTT, partial thromboplastin time; TCT, thrombin clotting time.

a Differentiating between vitamin K deficiency and liver disease can be challenging with conventional laboratory tests. If available, performing a factor II assay with and without Echis venom (factor II biologic and factor II Echis) may be useful. Ecarin is derived from Echis carinatus snake venom and can activate prothrombin irrespective of γ-carboxylation. Factor II activity (biologic) is reduced in both vitamin K deficiency and liver disease. In contrast, the factor II Echis is reduced in liver disease but is normal in vitamin K deficiency.

From Hoffman R et al: Hematology: basic principles and practice, ed 7, Philadelphia, 2018, Elsevier.

Figure E1 Red Blood Cell Morphology in Liver Disease

A, Macrocytes have a Mean Corpuscular Volume Greater than 100 Fl and Can Be Oval-Shaped. Commonly Associated Disorders Include Liver Disease and Vitamin B12 and Folate Deficiency. B, Target Cells are Characterized by the Bull’s-Eye Appearance of the Red Blood Cell (RBC). They are a Result of an Increased Surface-to-Volume Ratio Related to Excess RBC Membrane (E.g., Liver Disease) or Disproportionate Reduction of Cytoplasmic Content (E.g., Hemoglobinopathies, Iron Deficiency). C, Acanthocytes, or Spur Cells, Typically Appear as Contracted RBCs Lacking Central Pallor with Multiple Irregular Membrane Projections. The Morphologic Appearance Reflects the Irreversible Cytoskeletal Damage that Has Occurred Because of Passage of Nondeformable RBCs through the Reticuloendothelial System. They are Most Commonly Seen in Severe Liver Disease but Can Also Be Features of Rare Neuroacanthocytosis Syndromes or Lipoprotein Disorders.

From Hoffman R et al: Hematology: basic principles and practice, ed 7, Philadelphia, 2018, Elsevier.

Imaging Studies

  • Ultrasonography is the procedure of choice. It can identify the size and shape of the liver (generally small and nodular in advanced cirrhosis) and can detect gallstones and dilation of bile ducts. The use of sonography on a periodic basis to screen for hepatocellular carcinoma in patients with cirrhosis should be considered.
  • CT scan (Figs. 2 and 3) is useful for detecting mass lesions in liver and pancreas, assessing hepatic fat content, identifying idiopathic hemochromatosis, diagnosing Budd-Chiari syndrome early, assessing dilation of intrahepatic bile ducts, and detecting varices and splenomegaly. However, ultrasound is generally the preferred imaging modality of choice.
  • MRI can be used to identify hemangiomas, hepatocellular carcinoma.
  • Vibration-controlled transient elastography and magnetic resonance elastography are useful to assess advanced fibrosis. These noninvasive tests are less expensive and safer than biopsy and can be repeated over time to monitor disease progression.
  • Technetium-99m sulfur colloid scanning is rarely used but can be useful for diagnosing cirrhosis (there is a shift of colloid uptake to the spleen and bone marrow), identifying hepatic adenomas (cold defect is noted), and diagnosing Budd-Chiari syndrome (there is increased uptake by the caudate lobe).
  • Endoscopic retrograde cholangiopancreatography can be used for diagnosing periampullary carcinoma and common duct stones; it is also useful in diagnosing primary sclerosing cholangitis.
  • Percutaneous transhepatic cholangiography is useful when evaluating patients with cholestatic jaundice and dilated intrahepatic ducts by ultrasonography; presence of intrahepatic strictures and focal dilation is suggestive of primary sclerosing cholangitis.
Figure 2 Advanced Cirrhosis with Fatty Infiltration

Delayed Portal Venous-Phase Computed Tomography Reveals that the Liver is Misshapen and Nodular in Contour. Parenchymal Density that is Significantly Lower than that of the Spleen (S) is Indicative of Fatty Infiltration and Continuing Liver Injury. Prominent Scars and Bands of Fibrosis (Arrowheads) are Seen Throughout the Liver. Ascites (a) is Present. (From Webb Wr Et Al: Fundamentals of Body CT, Ed 4, Philadelphia, 2015, Saunders.)

Figure 3 Cirrhosis with Portal Hypertension

Postcontrast Computed Tomography Reveals a Liver that is Nodular in Contour (Arrowhead) with Patent Enlarged Paraumbilical Veins (Blue Arrows) and Splenomegaly (S), Findings Indicative of Portal Hypertension. Mildly Enlarged Portosystemic Collateral Vessels (Curved Arrow) are Also Evident in the Gastrohepatic Ligament.

From Webb WR et al: Fundamentals of body CT, ed 4, Philadelphia, 2015, Saunders.

Treatment ⬆ ⬇

Nonpharmacologic Therapy

  • Avoid any hepatotoxins (e.g., ethanol, acetaminophen), improve nutritional status, vaccinate against hepatitis A and B if not already immune.
  • Transjugular intrahepatic portosystemic shunt (TIPS, Fig. E4) in patients with recurrent variceal hemorrhage despite optimal medical therapy. Early use of TIPS is associated with significant reductions in treatment failure and in mortality in patients with cirrhosis who are hospitalized for acute variceal bleeding and are at high risk for treatment failure.
  • Correction of malnutrition: Daily protein intake of 1.0 to 1.5 g per kg of body weight.

Figure E4 Transjugular Intrahepatic Portosystemic Shunt (TIPS)

This Shunt is Performed by Interventional Radiologists and Consists of an Expandable Metal Stent (Most Often Coated with Polytetrafluoroethylene) that Connects a Branch of the Portal Vein (High-Pressure Vein) to a Branch of the Hepatic Vein (Low-Pressure Vein). The Shunt Decompresses the Portal System and Portosystemic Collaterals, and Therefore It is Used in the Treatment of Selected Patients with Cirrhosis and Variceal Hemorrhage. The Shunt Also Decompresses the Hepatic Sinusoids and Therefore is Also Used in the Treatment of Refractory Ascites.

From Goldman L, Schafer AI: Goldman-Cecil medicine, ed 26, Philadelphia, 2019, Elsevier.

Acute General Rx

  • Nonselective beta blockers (nadolol, propranolol) in patients with cirrhosis and variceal hemorrhage, and some without hemorrhage but with high-risk bleeding features. Use with caution in patients with severe alcoholic hepatitis and decompensated cirrhosis with refractory ascites. Beta-blockers should be temporarily discontinued in patients with spontaneous bacterial peritonitis due to increased mortality and hepatorenal syndrome incidence.
  • Pruritus due to liver disease may be treated with cholestyramine 4 g/day initially. Dose can be increased to 24 g/day as needed.
  • Pain management: Avoid opiates (may precipitate or aggravate hepatic encephalopathy) and NSAIDs (increased risk of gastrointestinal bleeding and renal failure). Low-dose tramadol and lidocaine patches are generally well tolerated.
  • Sedatives: Benzodiazepines (lorazepam or oxazepam) may be used for alcohol withdrawal but should be avoided in patients with hepatic encephalopathy. Avoid benzodiazepines with liver metabolites (diazepam, chlordiazepoxide).
  • Statins: Can be safely started and continued in patients with hyperlipidemia and/or nonalcoholic fatty liver disease.
  • Proton pump inhibitors: Not routinely indicated; their use in patients with cirrhosis is associated with excess risk for spontaneous bacterial peritonitis and hepatic encephalopathy; avoid indiscriminate use of proton pump inhibitors (PPIs) in patients with cirrhosis.
  • Antibiotics: Trimethoprim-sulfamethoxazole or ciprofloxacin/norfloxacin for spontaneous bacterial peritonitis prophylaxis in patients with a history of spontaneous bacterial peritonitis (SBP) an ascites protein concentration less than 1 g/dl, or history of variceal hemorrhage.
  • Liver transplantation may be indicated in otherwise healthy patients (ages <65 yr) with severe cirrhosis and lack of contraindications. Contraindications to liver transplantation are AIDS, most metastatic malignancies, active substance abuse, uncontrolled sepsis, and uncontrolled cardiac or pulmonary disease.
  • Complications of cirrhosis (Fig. 5): Causes of hepatic decompensation in cirrhosis are summarized in Box E1. Treatment of complications of portal hypertension (ascites, esophagogastric varices, hepatic encephalopathy, spontaneous bacterial peritonitis, and hepatorenal syndrome; refer to these individual topics in Section I).
  • Fig. 6 summarizes the management of compensated and decompensated cirrhosis.
  • Box 2 summarizes the management of coagulopathy in liver disease.

BOX 2 Management of Coagulopathy in Liver Disease

  • Actively bleeding patients should be adequately resuscitated. Admission to the intensive care setting may be appropriate.
  • Basic coagulation tests should be ordered to identify the cause of bleeding; these include CBC, PT (INR), PTT, thrombin clotting time, fibrinogen, D-dimer, FDP, and mixing studies. The need for more specialized tests will be dictated by the clinical situation and response to therapy.
  • It is important to identify any localized source of bleeding (e.g., varices) amenable to procedural intervention to achieve hemostasis.
  • A trial of 5-10 mg of vitamin K is reasonable in asymptomatic patients with prolonged PT and PTT but should be used with other therapies in actively bleeding patients.
  • In patients with thrombocytopenia, platelet transfusions can be used, targeting platelet counts greater than 50 × 109 /L.
  • In patients who can tolerate volume, FP 4-6 units (1000-1500 ml) given over 1-2 hr can be used to replace coagulation factors. Coagulation parameters should be monitored to document effect and determine the timing and need for additional units.
  • Dysfibrinogenemia or hypofibrinogenemia should be suspected if coagulation assays do not correct with FP or fibrinogen levels are low, respectively. Replacement can be attempted with 10-20 units of cryoprecipitate while following laboratory results.
  • Patients who are intravascularly overloaded or who do not respond to FP should be considered for rFVIIa. Low doses of rFVIIa (25-50 μg/kg) are generally used, and repeated doses may be required because of the short rfVIIa half-life of 2-3 hr. rFVIIa may be most suitable as a temporizing measure to enable invasive procedures or hemostasis to be achieved by other means. Avoid use in the setting of DIC.

From Hoffman R et al: Hematology: basic principles and practice, ed 7, Philadelphia, 2018, Elsevier.

Figure 6 Summary of the Management of Compensated and Decompensated Cirrhosis

Afp, -Fetoprotein; Bm, Bowel Movement; D/C, Discontinue; Egd, Esophagogastroduodenoscopy; GI, Gastrointestinal; HCC, Hepatocellular Carcinoma; INR, International Normalized Ratio; Na, Sodium; NSAIDs, Nonsteroidal Antiinflammatory Drugs; R/O, Rule Out; Sbp, Spontaneous Bacterial Peritonitis; Us, Ultrasound.

!!flowchart!!

From Goldman L, Schafer AI: Goldman-Cecil medicine, ed 26, Philadelphia, 2019, Elsevier.

Figure 5 Complications of Cirrhosis Result from Portal Hypertension or Liver Insufficiency

Varices and Variceal Hemorrhage are a Direct Consequence of Portal Hypertension. Ascites Results from Sinusoidal Portal Hypertension and Can Be Complicated by Infection (Spontaneous Bacterial Peritonitis [sbp]) or Renal Dysfunction (Hepatorenal Syndrome [HRS]). Hepatic Encephalopathy Results from Portosystemic Shunting (I.e., Portal Hypertension) and Liver Insufficiency. Jaundice Results Solely from Liver Insufficiency.

!!flowchart!!

From Goldman L, Schafer AI: Goldman-Cecil medicine, ed 26, Philadelphia, 2019, Elsevier.

BOX E1 Causes of Hepatic Decompensation in Cirrhosis

Infection

UTI, SBP, pneumonia, primary bacteremia, cellulitis

Metabolic

Renal failure, hyponatremia, uremia, volume depletion (variceal bleed)

Drugs

Alcohol, acetaminophen (paracetamol) especially

Also NSAIDs, diuretics, benzodiazepines, opiates, phenothiazines, anticonvulsants

Viral infection

Hepatitis A, B, C, D; cytomegalovirus, Epstein-Barr virus

Malignancy

HCC, cholangiocarcinoma, metastasis

Vascular

Portal vein thrombosis, Budd-Chiari syndrome

From Talley NJ et al: Essentials of internal medicine, ed 4, Chatswood, NSW, 2021, Elsevier Australia.

Treatment Based On Specific Cause Of Cirrhosis

  • Remove excess body iron with phlebotomy and deferoxamine in patients with hemochromatosis.
  • Remove copper deposits with D-penicillamine in patients with Wilson disease.
  • Long-term ursodiol therapy will slow the progression of primary biliary cholangitis. It is, however, ineffective in primary sclerosing cholangitis.
  • Glucocorticoids (prednisone 20 to 30 mg/day initially or combination therapy or prednisone and azathioprine) are useful in autoimmune hepatitis.
  • Antivirals in chronic hepatitis C.
Prognosis

  • Prognosis varies with the etiology of the patient’s cirrhosis and whether there is ongoing hepatic injury. Fig. 7 illustrates the natural history of cirrhosis.
  • Patients with compensated cirrhosis (no associated complications or esophageal varices without bleeding) have a good prognosis with median survival over 12 yr.
  • In patients with decompensated cirrhosis, the Model of End-stage Liver Disease with sodium (MELDNa) scoring (Table 3) is useful to predict 3-mo mortality and so is the main method of prioritizing patients awaiting liver transplantation. It also has prognostic value following TIPS placement for patients with cirrhosis undergoing nontransplant surgeries. Mortality rate exceeds 80% in patients with hepatorenal syndrome.
  • Regression of cirrhosis has been demonstrated after antiviral therapy in some patients with chronic hepatitis C. Regression is associated with decreased disease-related morbidity and improved survival.
  • Cirrhosis is associated with an increased risk for hepatocellular carcinoma. However, the risk is low (1% 5-yr cumulative risk in alcoholic cirrhosis).

TABLE 3 The Two Most Commonly Used Scoring Systems in Cirrhosis

1. Child-Turcotte-Pugh (CTP) Score (Range, 5-15)
Points Ascribed
Parameters123
AscitesNoneGrade 1-2 (or easy to treat)Grade 3-4 (or refractory)
Hepatic encephalopathyNoneGrade 1-2 (or induced by a precipitant)Grade 3-4 (or spontaneous)
Bilirubin (mg/dl)<22-3>3
Albumin (g/dl)>3.52.8-3.5<2.8
Prothrombin time (seconds >control) or INR<4
<1.7		4-6
1.7-2.3		>6
>2.3
CTP classification: Child A: Score of 5-6; Child B: Score of 7-9; Child C: Score of 10-15
2. Model of End-Stage Liver Disease (MELD) Score (Range, 6-40)
[0.957 × LN (creatinine in mg/dl) + 0.378 × LN (bilirubin in mg/dl) + 1.12 × LN (INR) + 0.643] × 10
MELD-Na is calculated first by determining the traditional MELD (MELD(0)); if the initial MELD(i) score is 12 or greater, the score is adjusted by incorporating the serum sodium value [MELD(0) + 1.32 × (137-Na) – [0.033 × MELD(i) × (137-Na)]

INR, International normalized ratio; LN, natural logarithm.

From Goldman L, Schafer AI: Goldman-Cecil medicine, ed 26, Philadelphia, 2019, Elsevier.

Figure 7 Natural History of Cirrhosis

Any Chronic Liver Disease Will Lead to Cirrhosis. Initially, Cirrhosis Will Be Compensated (Median Survival, >12 Yr), but Once Complications (Ascites, Variceal Hemorrhage, Encephalopathy, Jaundice) Develop, It Becomes Decompensated (Median Survival, 1.6 Yr). Hepatocellular Carcinoma (HCC) Can Develop at any Stage and Precipitate Decompensation and Death.

!!flowchart!!

From Goldman L, Schafer AI: Goldman-Cecil medicine, ed 26, Philadelphia, 2019, Elsevier.

Pearls & Considerations ⬆ ⬇

Comments

  • Thrombocytopenia and advanced Child-Pugh classes (see Table 3) are associated with the presence of varices. These factors are useful to identify cirrhotic patients who benefit most from referral for endoscopic screening for varices.
  • A combination of endoscopic and drug therapy reduces overall and variceal rebleeding in cirrhosis more than either therapy alone.
  • PPI use, but not H2RA use, is associated with risk for serious infections in patients with decompensated cirrhosis.
  • In patients with compensated cirrhosis and clinically significant hypertension beta-blockers reduced a composite of decompensation or death.
  • Patients with cirrhosis require pneumococcal vaccination, influenza, herpes zoster, tDAP, MMR, varicella, and hepatitis A and B vaccination.1
Related Content

Cirrhosis (Patient Information)

Ascites (Related Key Topic)

Esophageal Varices (Related Key Topic)

Hepatic Encephalopathy (Related Key Topic)

Hepatopulmonary Syndrome (Related Key Topic)

Hepatorenal Syndrome (Related Key Topic)

Spontaneous Bacterial Peritonitis (Related Key Topic)

Primary Biliary Cholangitis (Related Key Topic)

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