Budd-chiari Syndrome in a 25-year-old Woman with Behçet's Disease

A Case Report and Review of the Literature

Daniela T Carvalho; Fernando T Oikawa; Nilce M Matsuda; Paulo RB Évora; Alice T Yamada


J Med Case Reports. 2011;5(1) 

In This Article

Case Presentation

A 25-year-old Afro-Brazilian woman was hospitalized in a public hospital with the following complaints: ascites, dyspnea after exercise, and the development of veins and edema in the abdominal wall and swelling in the legs. Five years earlier, she had developed asymmetric recurrent migratory arthritis in her wrists and ankles, moderate and intermittent fever, recurrent painful ulcers and lesions in the oral cavity and vagina, and painful transient erythema nodosum on her forearm and legs. She reported recurrent erysipelas, light smoking and moderate alcoholism. She denied abortion, use of oral contraceptives and a pathological family history.

Physical examination showed that the patient had mild dyspnea, jaundice, pale skin, absence of fever and jugular turgescence, adenopathy, acneiform eruptions on the face and trunk, reduced vesicular murmur at right lung base, ascites with varicose veins in the abdomen near the skin surface, an enlarged and tender liver and edema of legs (++/4). She developed a rapid increase in the abdominal volume, abdominal pain and dyspnea after exercise and onset of jugular turgescence.

Laboratory tests detected hypochromic and microcytic anemia; nonreactive viral hepatitis serology; nonreactive HIV and syphilis infection serology; negative autoantibodies; undetected rheumatoid factor and serum complement; normal levels of protein C, S, and antithrombin II; high hemosedimentation velocity and C-reactive protein; serum ascites albumin gradient greater than 1.1; normal indirect binocular ophthalmoscopy; and a positive skin pathergy test. A vaginal histopathology of the lesion showed nonspecific chronic inflammatory process. The chest radiograph and computed tomography examination of the patient showed pleural effusion on the right pulmonary base (Figure 1). An echocardiogram showed no pulmonary hypertension but mild systolic deficit by diffuse hypokinesia of the left ventricle, pulmonary artery pressure of 25 mm Hg, ejection fraction of 40% and mild pericardial effusion. Doppler ultrasound examination of suprahepatic and cava veins showed absence of flow in the left suprahepatic vein.

Figure 1.

Chest computed tomography (CT) examination of the patient. This chest CT showed pleural effusion on the right pulmonary base.

Symptomatic treatment was established and added antibiotic therapy and use of angiotensin-converting enzyme inhibitors, diuretics and a methylprednisolone pulse therapy followed by oral corticosteroids, azathioprine, colchicine, anticoagulants and methotrexate (replacing the colchicine at hospital discharge for better convenience of administration). After such treatment was begun, the patient had significant improvement. The patient was discharged from the hospital and returned to the outpatient clinic twice - the first 15 days and the second 45 days after hospital discharge; in both outpatient consultations, the patient was well.