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Acute and fulminant Budd-Chiari syndrome in a well-anticoagulated patient with primary antiphospholipid syndrome.

Sir – Antiphospholipid syndrome (APS) is a complex entity defined by the presence of antibodies to negatively charged cellular membrane phospholipids. Obstetric complications, such as miscarriages or recurrent fetal losses and thrombotic events, both arterial and venous, are the most common features of this condition.1,2 Deep vein thrombosis predominantly affects the lower limbs in patients with APS. Budd– Chiari syndrome (BCS) is an infrequent complication among APS patients and sometimes is the first manifestation of the disease.3,4 We describe a patient with APS and BCS, with an atypical and fatal outcome, that occurred when the patient was properly anticoagulated with acenocumarole as recommended.5,6 A 77-year-old woman was admitted to the hospital because of abdominal pain and distension over the previous five days. She suffered from hypertension, hypothyroidism and chronic venous insufficiency. She had been diagnosed with primary APS four years before on the basis of the presence of antiphospholipid antibodies (lupus anticoagulant at high titre and antiprothrombine IgM level of 120 U) and ischaemic neurologic events with multiple brain infarcts and cerebellous atrophy on the neuroimaging study. Antinuclear antibodies, rheumatoid factor and myeloperoxidase neutrophil cytoplasmic antibodies (ANCA, anti-MPO) were also positive. The anticardiolipin antibodies were negative. She attended to the Internal Medicine Department regularly and anticoagulation rates were within the therapeutic range (INR 2.5–3.5). Three days before admission, diffuse abdominal pain and distension started. She complained of loss of appetite, vomiting three weeks before, with no constipation or diarrhoea. On physical examination, muco-cutaneous dryness, slight conjunctival jaundice and distension of the jugular veins with positive abdominojugular reflux were observed. Cardiac examination showed atrial fibrillation with no murmurs, and the lungs were clear on pulmonary examination. Abdominal examination showed severe distension with signs of ascites and collateral circulation. A 3 cm tender hepatomegaly was also noted. Lower limbs appeared normal. Neither Raynaud’s phenomenon nor livedo reticularis were observed. Tests for antibodies to hepatitis B, C and HIV were negative. A diagnostic and therapeutic paracentesis was performed, showing a trasudate, with low values for proteins, adenosine deaminase and lactic dehydrogenase. The cytologic examination was negative for malignant cells. A Gram’s stain showed mononuclear cells, and culture rendered no growth of bacteria. A smear and culture were negatives for fast acid–alcohol bacilli. Abdominal ultrasound showed ascites with portal hypertension signs. An abdominal computed tomography (CT) scanning disclosed hepatomegaly with a markedly disrupted hepatic perfusion pattern. The suprahepatic and intrahepatic cava were less visible and had no contrast refill. The porta was permeable, splenomegaly and ascites were visible. All of these traits were compatible with a BCS. During admission coagulation markers worsened progressively, with important increase in INR values to as much as 12.3, even though anticoagulation therapy had been stopped. Plasmapheresis, prednisolone and cyclophosphamide pulses were started. An improved consciousness was shown although impaired renal function remained: creatinine levels increased from 1.6 to 4.9 mg/dL. A transjugular intrahepatic portosystemic shunt (TIPS) was tried unsuccessfully, so a surgical shunt was performed on the 14th day. Post-surgical evolution was unfavourable: oligoanuria persisted despite inotropic agents, volume expanders, fresh frozen plasma and plasmapheresis sessions; hepatic encephalopathy, severe coagulopathy and hepatorenal syndrome developed, and, three days after surgery, the patient died. Postmortem examination was refused. BCS has been previously observed as a part of the wide thrombotic spectrum of APS.3,4,7 Espinosa et al. described the clinical and immunological characteristics of 43 patients with BCS and APS.4 The mean age was 30.8 years, much younger than the patient described. The clinical onset of our case was similar to that described previously. However, a fulminant evolution with oligoanuric renal failure, coagulopathy, intractable ascites and hepatorenal syndrome was present in only 7% of the patients. The clinical evolution was subacute in 70% and chronic in 23% of the patients. Two out of three fulminant cases occurred during postpartum. Doppler ultrasonography of the liver is the technique of choice for the initial investigation and is a diagnostic tool in most of the cases.