Occurrence of Chronic Budd-Chiari Syndrome in a Patient with Coeliac Disease

The association of Budd-Chiari Syndrome (BCS) with coeliac disease has been understudied and underreported. Majority of the cases of this association have been reported from North Africa and Southern Europe, due to a higher prevalence of coeliac disease. BCS can present as acute, subacute, or chronic form and is characterised by hepatic venous obstruction, either thrombotic or non-thrombotic without any evidence of sinusoidal obstruction syndrome, right heart failure or constrictive pericarditis. Unlike patients with BCS alone, an underlying hypercoagulable state cannot be identified in most patients if coeliac disease is associated with BCS. Its incidence is found to be low in the rest of the world. Here, we present a case of coeliac disease and BCS from an Indian setting in a patient who was previously diagnosed with coeliac disease six years back, maintained on a gluten-free diet, and now presented with complaints of chronic Budd-Chiari Syndrome without any identifiable hypercoagulable aetiology.