Introduction
Budd–Chiari Syndrome (BCS) is a rare disorder typically acquired rather than congenital. It is characterized by an obstruction within the hepatic venous outflow tract, occurring anywhere from the terminal hepatic veins to the confluence of the inferior vena cava (IVC) and the right atrium. This obstruction leads to hepatic dysfunction. The diagnosis of primary BCS is established when obstruction primarily results from a vascular pathology, including thrombosis or phlebitis. Conversely, secondary BCS is identified when external compression or invasion of the hepatic veins and/or inferior vena cava (IVC) is induced by a lesion, such as malignancy 1,2.
George Budd is credited with the initial delineation of a clinical syndrome characterized by the occlusion of hepatic veins, BCS, which was documented in 1846 3. Primary myeloproliferative diseases stand out as the predominant causative factor for the onset of BCS. Clinically discernible disease manifestation requires the occlusion of at least two hepatic veins 4.
The majority of BCS patients present with the classic triad of abdominal pain in the upper right quadrant presenting in about 61% of cases, hepatomegaly presenting in 67% of cases and ascites presenting in 83% of cases 5,6. Advanced BCS leads to the development of portal hypertension and liver failure, accompanied by complications such as encephalopathy and hematemesis 7. BCS should be considered in both symptomatic and asymptomatic individuals with acute or chronic liver disease. Recent guidelines emphasize the crucial role of non-invasive imaging for early BCS identification and assessment, which include Doppler ultrasound, Magnetic resonance imaging (MRI), and computed tomography (CT) 1,8.
Survival rates in patients with Budd–Chiari syndrome have significantly improved due to advancements in endoscopic technology, Transjugular Intrahepatic Portosystemic Shunt (TIPSS), and liver transplantation, with reported survival rates reaching 96%9. However, a large proportion of cases go undiagnosed due to nonspecific symptoms and delayed diagnosis.
Adrenal insufficiency poses a life-threatening risk arising from either primary adrenal failure or secondary adrenal disease, often attributed to dysfunction in the hypothalamic-pituitary axis. This condition manifests clinically as the inadequate production or effectiveness of glucocorticoids, sometimes accompanied by a deficiency in mineralocorticoids and adrenal androgens. Hydrocortisone serves as the cornerstone in the therapeutic approach for addressing adrenal insufficiency. It is also documented to be associated with a procoagulant state leading to an increased risk of venous thrombosis and thrombo-embolism 10,11.
Herein, we present a unique instance of Budd-Chiari syndrome occurring in a long-term hydrocortisone user with Addison’s insufficiency, marking the first documented case of its kind.
This case report is in line with the CARE guidelines for case reports12. The patient provided all the data used for the case report and consent to the study.