Discussion:
The above case report presented the rare occurrence of Budd Chiari Syndrome (BCS) in a patient who was previously diagnosed with Addison’s disease. To the best of our knowledge, this case is the first of its kind to be reported.
Addison’s disease is a primary adrenal insufficiency caused by autoimmune mechanisms, causing bilateral adrenal cortex destruction, thereby reducing the production and release of adrenocortical hormones. Patients with adrenal insufficiency have been documented to be at a higher risk of death due to infections, cancer, and cardiovascular causes 13. Various case reports have documented cardiovascular complications in patients previously diagnosed with Addison’s disease. A case report by Zhao et.al presented a unique case of coronary artery disease in a patient with Addison’s disease, where Addison’s disease was attributed to speeding up the progression of CAD and causing chest pain. Glucocorticoids provided as therapeutic agents for Addison’s disease were thought to have caused these vascular complications 14. Similarly, cases of cardiomyopathy15 and heart failure 16 have also been documented in patients diagnosed with Addison’s disease.
The incidence of Budd–Budd-Chiari syndrome is sparsely documented in the literature, limited to a handful of studies owing to the rarity of the disease. The incidence of this syndrome in the general population is 1 in 1,00,000. A hypercoagulable state is detectable in 75% of patients, and in 25% of cases, the involvement of more than one etiologic factor is evident 2. Doppler ultrasound, with a sensitivity and specificity exceeding 85%, serves as the primary investigative tool. Magnetic resonance imaging (MRI) and computed tomography (CT) are reserved for diagnostic confirmation in specific cases. Doppler sonography reveals elevated flow velocities with turbulence at the stenosis level and reduced flow proximally during breathing phases. Intrahepatic collaterals, extending from occluded to non-occluded hepatic veins, are a specific diagnostic criterion8. In managing BCS, the primary objective is to restore venous flow irrespective of obstruction location. A stepwise approach, tailored to the varied etiological factors, guides the progression from one technique to another, ensuring adherence to key treatment criteria. Without treatment, the 3-year mortality rate is 90%3.
The medical management strategy for patients with Budd-Chiari Syndrome (BCS) involves promptly initiating anticoagulant therapy for an indefinite duration. This approach aims to mitigate the risk of new thrombotic episodes and prevent the extension of existing clots. As per the guidelines for deep vein thrombosis, it is advised to initiate treatment with low molecular weight heparin (LMWH) for a duration of at least 5 to 7 days. Simultaneously, oral anticoagulant therapy with a Vitamin K antagonist should be commenced, with the goal of achieving an international normalized ratio (INR) ranging between 2 and 3. The administration of LMWH can be discontinued once the INR consistently falls within the target range for two consecutive measurements8. In the treatment protocol for Budd-Chiari Syndrome (BCS), a liver transplant is considered the final resort. Despite medical management, percutaneous revascularization, and trans jugular intrahepatic portosystemic shunt (TIPSS), around 10%‐20% of BCS patients experience ongoing liver deterioration. For these individuals, a liver transplant emerges as the only remaining therapeutic option. Additionally, a liver transplant is the preferred treatment for selected BCS cases in whom hepatocellular carcinoma (HCC) develops and patients meet the transplantation criteria 17.
Although the incidence of BCS in patients with Addison’s disease has not been documented, there have been incidences of patients presenting with BCS following prolonged intake of corticosteroids and contraceptive steroid medications 18,19. Mederacke et.al. reported a case of a 40-year-old cirrhotic patient who was diagnosed with BCS after treatment with Budesonide, indicating that the administration of Budesonide in patients with established cirrhosis may increase the risk of serious complications 18. Budesonide has been suggested as a treatment for primary biliary cholangitis (PBC). However, in a trial published by Hempfling et.al., it was noted that plasma levels of budesonide were higher in PBC stage IV compared to stages I/II. Significantly, two out of seven patients with PBC stage IV experienced a serious complication of portal venous thrombosis20. Cases of BCS associated with the intake of steroidal oral contraceptive pills have also been documented, implicating these drugs as the cause of many instances of hepatic vein thrombosis 19.McDow et al. 21 have demonstrated that the occurrence of portal vein thrombosis in individuals with newly diagnosed Cushing’s syndrome suggests that there may be an underlying hypercoagulable state associated with it. This suggests that it is reasonable to suggest that chronic long-term hydrocortisone use can be associated with an underlying hypercoagulable state. This case highlights the significance of prompt intervention to prevent complications in Budd-Chiari Syndrome and suggests a potential association between Budd-Chiari Syndrome and long-term hydrocortisone use.