A unique combination of celiac disease, mesenteric lymph node cavitation, splenic atrophy and necrotizing hepatitis

We report on a patient who was diagnosed six years before with celiac disease, with a current combined problem of asplenism, mesenteric cysts and elevated liver function tests. The implications of splenic atrophy mimic those of post-splenectomy patients. Mesenteric lymph node cavitation is a rare complication of celiac disease that is most often associated with splenic atrophy. The pathogenesis is unknown. The clinical implications of the cavitated mesenteric lymph nodes are unclear. The association of celiac disease with liver disease was reported many years ago, but only recently these associations have been more clearly defined. Liver involvement shows a clinical spectrum varying from nonspecific reactive hepatitis, chronic active hepatitis, steatohepatitis to frank cirrhosis. Associations with autoimmune hepatitis, autoimmune cholangitis, primary biliary cirrhosis and primary sclerosing cholangitis have been described. In our patient, we found no obvious cause for the necrotizing hepatitis and the negative auto-antibodies made it impossible to firmly establish the diagnosis of autoimmune hepatitis. The causal relationship with celiac disease, if any, remains unproven.