A 32-year-old female patient presented with progressive abdominal distension of four months. She had also right upper quadrant abdominal pain, low-grade intermittent fever, and night sweating of the same duration, along with a weight loss of 6 kg over a 4-month period. For these complaints, she visited a nearby hospital, and with the impression of peritoneal tuberculosis, she was started on antituberculosis drugs, but there was no improvement. The patient had no history of oral contraceptive use or recurrent pregnancy loss. The patient tested negative for HIV. The patient had no history of diabetes mellitus, hypertension, alcohol consumption, or cigarette smoking.
Upon physical examination, she was well looking, with a blood pressure of 100/75 mmHg, pulse rate of 80 beats per minute, respiratory rate of 18 breaths per minute, and body temperature of 37.1  C. She had pink conjunctivae and non-icteric sclerae. Abdominal examination revealed a grossly distended abdomen that moved with respiration, with positive shifting dullness and fluid thrill. No abdominal tenderness or palpable organomegaly was noted.
She had a complete blood count of 4800 cells/microL, hemoglobin of 14.6 g/dL, and platelet count of 227,000 cells/microL. Liver enzyme, serum albumin, and bilirubin levels were within normal ranges. Tests for hepatitis B and C viral markers yielded negative results. Ascitic fluid analysis showed a white blood cell count of 250 cells/microL, protein of 4.1 g/dL, glucose of 106 g/dL, negative gram stain and negative AFB. The serum ascitic albumin level gradient (SAAG) was 1.3. Cytology of the ascitic fluid was significant for lymphocytosis, but no malignant cells were observed.
Abdominal ultrasonography revealed a liver size of 16 cm with mild coarse echo texture, an enlarged caudate lobe, nonvisualized hepatic veins, and free fluid collections within the peritoneum. On the abdominal CT scan, the left and middle hepatic veins were not visualized; the right hepatic vein and intrahepatic IVC had luminal narrowing, and there was caudate lobe enlargement (Figures 1 and 2). Upper gastrointestinal endoscopy revealed grade 1 esophageal varices.  
After the diagnosis of Budd-Chiari syndrome (BCS) was confirmed by abdominal computed tomography (CT), the patient was started on anticoagulation therapy with unfractionated heparin overlapped with warfarin followed by warfarin alone. The patient was also started on diuretics (furosemide with spironolactone) and beta blocker (propranolol 20mg PO BID). During follow-up, she showed marked symptomatic improvement with a decrease in the amount of ascites. However, after 6 months of BCS diagnosis, while on follow-up, she started to develop bilateral knee joint pain with swelling, alopecia, and oral lesions. After these manifestations developed, the patient was further investigated for systemic lupus erythematosus (SLE) and antiphospholipid (APS) and laboratory tests revealed positive antinuclear antibodies (ANA) with titer of 1:320, positive anti-double-stranded DNA (dsDNA) antibodies with level of 40 IU/mL (reference range: <30 IU/mL), and positive antiphospholipid antibodies (anticardiolipin antibodies and beta 2 glycoprotein 1 antibodies).
The patient was finally diagnosed with Budd-Chiari syndrome secondary to SLE associated with APS and was initiated on chloroquine phosphate 250mg PO 5 times per week and steroid (prednisolone), while warfarin, diuretics and propranolol were continued. The patient was counseled on the need of advanced therapies like transjugular intrahepatic portosystemic shunting (TIPS) placement, surgical shunting or liver transplantation if any worsening of the symptoms of BCS despite medical therapies.