Budd-Chiari Syndrome as an Initial Manifestation of Incomplete Systemic Lupus Erythematosus.

Abstract

This article describes a case of a 26-year-old female with a history of Evan's syndrome who presented with severe exertional dyspnoea and abdominal discomfort. The patient was diagnosed with chronic Budd-Chiari syndrome, a rare vascular disorder characterized by obstruction of the hepatic vein. We discuss the risk factors, the clinical manifestations, and diagnostic methods for Budd-Chiari syndrome, as well as the possible association with an underlying incomplete systemic lupus erythematosus. The importance of close follow-up and timely diagnosis for preventing disease progression and reducing mortality is emphasized. The article concludes by highlighting the need for further monitoring to identify any symptoms or signs suggesting a progression to complete lupus erythematosus.

Learning points: Budd-Chiari syndrome can present as the initial manifestation of a broader autoimmune disorder such as incomplete systemic lupus erythematosus.Evan's syndrome, when associated with other thrombotic conditions like antiphospholipid syndrome, may indicate an underlying, evolving autoimmune process.Early recognition and management of incomplete lupus are crucial to prevent progression to a full-blown systemic lupus erythematosus and associated complications.