Celiac disease is a common (1% prevalence) chronic immune-mediated disorder of the small intestine induced by dietary wheat, barley, and rye. Several hepatic disorders have been described in association with celiac disease. Isolated hypertransaminasemia with nonspecific histologic changes in a liver biopsy is the commonest hepatic presentation of celiac disease. A gluten-free diet normalizes liver enzymes and histologic changes in most patients. Moreover, celiac disease can coexist with autoimmune liver disorders such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis. Celiac disease has increasingly been reported with a variety of other liver diseases. Thus, the hepatologist needs to consider celiac disease in the differential of abnormal liver blood tests and to be aware of the clinical implications of this frequent disease in patients with liver disorders. The possible mechanisms of liver injury and those common factors that explain the association of celiac disease with liver disorders are discussed. The aims of this article are (1) to review the spectrum and pathogenesis of liver injury related to celiac disease and (2) to provide direction to those caring for patients with chronic liver diseases regarding the detection and effective treatment of celiac disease. (HEPATOLOGY 2007;46:1650-1658.) C eliac disease (CD), also known as gluten-sensitive enteropathy or celiac sprue, is defined as a permanent intolerance to ingested gluten (the storage protein components of wheat, barley, and rye). The intolerance to gluten results in immune-mediated damage to the mucosa of the small intestine characteristically inducing villous atrophy and crypt hyperplasia that resolve with the removal of gluten from the diet. Although CD is defined by the small intestine injury and resulting malabsorption, more recently it has been recognized to be a multisystem disorder that may affect other organs, such as the nervous system, bones, skin, heart, and, likely, the liver. CD, once thought to be rare, is now known to affect as much as 1% of the population. The clinical presentation of CD can vary from a classical malabsorption syndrome to more subtle atypical gastrointestinal manifestations (similar to irritable bowel syndrome) or extraintestinal presentations (for example, infertility, osteoporosis, and iron-deficiency anemia). CD can be clinically silent, often detected by serologic screening of those subjects at risk, with villous atrophy in the intestine. Finally, an individual may have a latent predisposition to CD, which is defined by a positive serology in the absence of villous atrophy on the small intestine. CD itself may injure the liver but also may modify the clinical impact of chronic liver diseases when they coexist. The aims of this review are (1) to explore the spectrum and pathogenesis of liver abnormalities described in CD and (2) to summarize the association between CD and various chronic liver disorders to provide a basis for a rational diagnostic and therapeutic approach that those who care for patients with liver disease can incorporate into practice.
Material and Methods (Review Criteria)