Chapter 9 Cirrhosis and portal hypertension
an overview
1 The major causes of cirrhosis include chronic hepatitis B, chronic hepatitis C, alcohol, hemochromatosis, and nonalcoholic steatohepatitis (NASH).
2 An etiologic classification of cirrhosis is more clinically relevant than a morphologic one (micronodular, macronodular, mixed), because the cause can be determined in most cases, and important management issues such as family counseling, vaccination, and specific therapy are best addressed once the cause has been determined.
3 Important and potentially life-threatening complications of cirrhosis include ascites, spontaneous bacterial peritonitis, variceal hemorrhage, hepatic encephalopathy, hepatorenal syndrome, hepatopulmonary syndrome, and hepatocellular carcinoma.
4 The Child–Turcotte–Pugh classification is useful in assessing prognosis and estimating the potential risk of variceal bleeding and operative mortality.
Cirrhosis
Definition
1. The word cirrhosis is derived from the Greek word kirrhos, meaning orange or tawny, and osis, meaning condition.
2. World Health Organization definition of cirrhosis is a diffuse process characterized by fibrosis and the conversion of normal liver architecture into structurally abnormal nodules that lack normal lobular organization.
4. This definition distinguishes cirrhosis from other types of liver disease that have either nodule formation or fibrosis, but not both. These hepatic disorders may be characterized by portal hypertension in the absence of cirrhosis. Nodular regenerative hyperplasia, for example, is characterized by diffuse nodularity without fibrosis, whereas chronic schistosomiasis is characterized by Symmers’ pipestem fibrosis with no nodularity.
Classification
1. Morphologic classification is less useful because of considerable overlap.
Micronodular cirrhosis, with uniform nodules less than 3 mm in diameter: causes include alcohol, hemochromatosis, biliary obstruction, hepatic venous outflow obstruction, jejunoileal bypass, and Indian childhood cirrhosis.
Micronodular cirrhosis, with uniform nodules less than 3 mm in diameter: causes include alcohol, hemochromatosis, biliary obstruction, hepatic venous outflow obstruction, jejunoileal bypass, and Indian childhood cirrhosis.
2. Etiologic classification is preferred.
This method of classification is the most useful clinically; by combining clinical, biochemical, genetic, histologic, and epidemiologic data, the likely etiologic agent can be ascertained.
The two most common causes of cirrhosis are excessive alcohol use and viral hepatitis. Table 9.1 lists the etiologic classification and tests used to determine the cause.
This method of classification is the most useful clinically; by combining clinical, biochemical, genetic, histologic, and epidemiologic data, the likely etiologic agent can be ascertained. The two most common causes of cirrhosis are excessive alcohol use and viral hepatitis. Table 9.1 lists the etiologic classification and tests used to determine the cause.
TABLE 9.1 Etiology and diagnostic workup of the common causes of cirrhosis
| Etiology | Diagnostic evaluation |
|---|---|
| Infection | |
| Hepatitis B | HBsAg, anti-HBs, anti-HBc, HBV DNA |
| Hepatitis C | Anti-HCV, HCV RNA |
| Hepatitis D | Anti-HDV |
| Toxins | |
| Alcohol | History, AST/ALT ratio, liver biopsy |
| Cholestasis | |
| Primary biliary cirrhosis | AMA, IgM, liver biopsy |
| Secondary biliary cirrhosis | MRCP, ERCP, liver biopsy |
| Primary sclerosing cholangitis | MRCP, ERCP, liver biopsy |
| Autoimmune | |
| Autoimmune hepatitis | ANA, IgG level smooth muscle antibodies, liver-kidney microsomal antibodies, liver biopsy |
| Vascular | |
| Cardiac cirrhosis | Echocardiogram, liver biopsy |
| Budd–Chiari syndrome | CT, US, MRI/MRA, |
| Sinusoidal obstruction syndrome | History of offending drug use, liver biopsy |
| Metabolic | |
| Hemochromatosis | Iron studies, HFE gene mutation, liver biopsy |
| Wilson disease | Serum and urinary copper, ceruloplasmin, slit lamp eye examination, liver biopsy |
| Alpha-1 antitrypsin deficiency | Alpha-1 antitrypsin level, protease inhibitor type, liver biopsy |
| NASH | Liver biopsy |
| Cryptogenic | Exclude NASH, drugs |
ALT, alanine aminotransferase; AMA, antimitochondrial antibodies; ANA, antinuclear antibodies; anti-HBc, antibody to hepatitis B core antigen; anti-HBs, antibody to hepatitis B surface antigen; anti-HCV, antibody to hepatitis C virus; anti-HDV, antibody to hepatitis D virus; AST, aspartate aminotransferase; CT, computed tomography; ERCP, endoscopic retrograde cholangiopancreatography; HBsAg, hepatitis B surface antigen; IgG, immunoglobulin G; IgM, immunoglobulin M; MRA, magnetic resonance angiography; MRCP, magnetic resonance cholangiopancreatography; MRI, magnetic resonance imaging; NASH, nonalcoholic steatohepatitis; US, ultrasonography.
Pathology
1. Gross examination: the liver surface is irregular, with multiple yellowish nodules; depending on the severity of the cirrhosis, the liver may be enlarged because of multiple regenerating nodules or, in the final stages, small and shrunken.
Clinical Features
1. Stigmata of chronic liver disease on physical examination (e.g., palmar erythema, spider telangiectasias)
2. Abnormal serum chemistry test results and hematologic indices (e.g., serum aminotransferases, bilirubin, alkaline phosphatase, albumin, prothrombin time, and platelet count)
3. Radiographic abnormalities (e.g., small, shrunken, nodular liver on ultrasound or computed tomographic [CT] examination)
A patient with cirrhosis may present with none, some, or all of the following findings:
