Budd-Chiari Syndrome as the Presenting Feature of Systemic Immunoglobulin Light Chain Amyloidosis

Budd-Chiari syndrome (BCS), characterised by hepatic venous outflow obstruction, is frequently associated with hypercoagulable states. Systemic immunoglobulin light chain (AL) amyloidosis, on the other hand, commonly presents with bleeding complications. The association between the two diseases is not common, but some cases have been described in the past. We report a case of a 58-year-old male who presented with lower limb oedema, abdominal distention and severe fatigue, ultimately diagnosed with BCS secondary to systemic AL amyloidosis. This case highlights the rare association between AL amyloidosis and BCS, likely precipitated by a nephrotic syndrome-induced hypercoagulable state, and emphasises the need for clinicians to consider systemic amyloidosis when investigating the aetiology of BCS, even in the absence of typical bleeding manifestations.

AL amyloidosis is typically associated with bleeding disorders, but rarely can present with thrombotic complications as well, specifically when associated with nephrotic syndrome.Budd-Chiari syndrome is a life-threatening condition which should be on the differential diagnosis of subacute liver failure.A thorough aetiological investigation is essential in a patient with Budd-Chiari syndrome, including consideration for potential causes of nephrotic syndrome and other less typical disease associations.