Chapter 7 - Wilson disease – liver pathology

Abstract

The liver in Wilson disease may demonstrate a wide range of damage patterns. Some patients may present almost no detectable microscopic pathology, while others display lesions consistent with fulminant hepatitis or acute liver failure. Most liver biopsy specimens show moderate to severe steatosis, variable degree of portal and/or lobular inflammation, and fibrosis eventually progressing to cirrhosis. Additional findings include liver cell degeneration and ballooning, Mallory hyaline bodies, liver cell necrosis, and glycogenation of periportal hepatocytic nuclei. None of the above lesions are specific for Wilson disease and should be interpreted in a wider medical context and particular clinical setting. The main message concerning liver pathology is that Wilson disease may be microscopically misinterpreted as many other liver diseases, including viral or autoimmune hepatitis, alcoholic/nonalcoholic steatohepatitis, toxic liver injury, cryptogenic cirrhosis, metabolic liver disease, and many others. The possibility of Wilson disease should be considered in all patients, especially young ones presenting unexplained liver diseases with many variable patterns of microscopic liver involvement.