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.