Recurrent Thrombotic Events after Catastrophic Antiphopholipid Syndrome

A 40-year-old man was transferred to our hospital in March 1994 for gross hematuria and decreased urine output. The patient exhibited chest pain and shortness of breath 2 weeks prior to the initial visit. These symptoms were relieved without particular management. Fever and chills developed thereafter followed by abdominal pain. The patient visited a local clinic and was managed for acute pancreatitis and gastritis for 1 week. However, his symptoms did not subside and urine output decreased progressively. On admission, he complained of severe headache, nausea, and both flank and back pain. Initial hemoglo-bin levels and platelet counts were 16.9 g/dL and 478 × 10 9 /L, respectively. In the course of 3 days, anemia and thrombocytopenia rapidly developed, with a hemoglo-bin level of 12.0 g/dL and a platelet count of 29 × 10 9 / L. Peripheral blood smear showed schistocytes suggesting hemolytic anemia. Initial blood urea nitrogen and creatinine levels were 69 and 6.0 mg/dL, respectively. Magnetic resonance imaging of the kidney revealed acute cortical necrosis. He also had blood tinged spu-tum, and the lung perfusion scan revealed perfusion defects in the right lower lobe, suggesting pulmonary embolism. Despite fluid therapy and the administration of diuretics, azotemia progressed rapidly over a 3-day period and he consequently underwent hemodialysis. Azotemia improved with increased urine output only after hemodialysis (Fig. 1). The serologic results showed the presence of lupus an-ticoagulant and IgM anticardiolipin antibody, and the patient was diagnosed with antiphospholipid syndrome (APS) with multiorgan failure. Retrospectively, his clinical presentation fits into the category of probable catastrophic APS (CAPS). However, neither intravenous heparin nor warfarin was used due to hemoptysis. Thereafter, he was admitted several times for coronary events and pulmonary infarction. Two months after the first attack, he was readmitted due to chest pain. The echocardiogram showed inferior and septal myo-cardial infarction and he was discharged with aspirin and clopidogrel. Three months later, he was readmitted due to chest pain, and a coronary angiogram showed right coronary artery focal stenosis (Fig. 2). He was lost to follow-up for 3 years and was readmitted to the hospital in 1997 for pleuritic chest pain. Chest computed which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Figure 1. Azotemia progressed rapidly with fluid and diuretics therapy. Urine output increased only after hemodialysis. HD, hemodialysis; Cr, creatinine.