A 34-year-old woman with primary antiphospholipid syndrome was admitted to the Gastroenterology Department of our hospital with fever, acute abdomen, watery diarrhea, and extremely high levels of inflammatory parameters. She had a history of left lower limb deep vein thrombosis and pulmonary embolism and was taking warfarin potassium. Acute gastroenteritis was suspected and an antibiotic was administered, but symptoms progressed. Abdominal ultrasonography showed occlusion of the left hepatic vein and the middle hepatic vein and her D-dimer level was high. Accordingly, Budd-Chiari syndrome was diagnosed and high-dose intravenous infusion of heparin was initiated. Her abdominal symptoms improved and the levels of inflammatory parameters and D-dimer decreased rapidly. It is known that antiphospholipid syndrome can be complicated by Budd-Chiari syndrome that usually occurs as subacute or chronic onset, but acute onset is rare. It is difficult to diagnose acute Budd-Chiari syndrome complicating antiphospholipid syndrome and this complication generally has a poor outcome. However, the present case can get early diagnosis and successful treatment with tight anticoagulant therapy.
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A Case of Acute Budd-Chiari Syndrome Complicating Primary Antiphospholipid Syndrome Presenting as Acute Abdomen and Responding to Tight Anticoagulant Therapy
Case Reports in Rheumatology
fig2:Doppler ultrasonography of the hepatic veins. (a) Middle hepatic vein (arrowhead ①) and right hepatic vein (arrowhead ②). (b) Blood flow is absent in parts of the middle hepatic vein (arrow) and increased blood flow is seen in the region from the middle hepatic vein to the right hepatic vein (circle).
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Acute gastroenteritis was diagnosed and cefmetazole was started, while warfarin was continued. However, her abdominal pain did not improve and the antibiotic was switched to meropenem. Dynamic CT showed a small amount of ascites in the perihepatic region and expansion of the poorly enhanced area in the lateral segment of the left hepatic lobe, suggesting ischemia of the left lobe (Figure 1(a)). Since patient's condition was worsened under oral anticoagulation with warfarin and stopped oral intake because of strong abdominal pain, anticoagulant therapy was switched to heparin sodium (10,000 units/day) and further investigation was performed on the 4th day from admission. Doppler ultrasonography showed reflux of blood in the left branch of the portal vein (Figure 1(b)), as well as no blood flow in the left hepatic vein, obstruction in the central part of the middle hepatic vein, and increased blood flow in the right hepatic vein (Figure 2). Because of high levels of D-dimer and C-reactive protein and the Doppler imaging findings, thrombotic occlusion of the middle hepatic vein and left hepatic vein was strongly suspected, and acute BCS was diagnosed. On the 6th day, anticoagulant therapy was intensified, with infusion of heparin sodium in the range of 15000–25000 units/day to maintain an APTT ratio of 4.0–5.0 which was set at twice the baseline value. Subsequently, contrast-enhanced MRI showed no enhancement of the left hepatic vein and probable occlusion of the middle hepatic at its confluence with the inferior vena cava (Figure 3). After intensification of anticoagulant therapy, the patient's fever and abdominal pain resolved, and the levels of inflammatory parameters and D-dimer decreased without thrombocytopenia. On the 17th day, administration of warfarin was resumed and the PT-INR was maintained within the target range of 2.5–3.0; thereafter, heparin was discontinued on the 27th day. After discharge, there was no evidence of portal hypertension or ascites and the abnormal reflux flow at the left portal vein disappeared by abdominal ultrasonography 5 months later (Figure 4). She got good outcome under warfarin use alone.
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