Multiple Thromboses in a Patient with Systemic Lupus Erythematosus after Splenectomy

Antiphospholipid syndrome is a disorder presenting with arterial or venous thrombus and a history of fetal loss. Early diagnosis and adequate treatment is important to prevent multiple organ failures. Here, we described a woman with a two-year history of systemic lupus erythematosus with severe nephrotic syndrome, manifested multiple thrombi over the portal vein and the inferior vena cava, combined with acute renal infarction. The patient underwent splenectomy 10 months ago. Initially, she received anticoagulant treatment and low-dose glucocorticoid, but multiple organ failure progressed. After emergency plasma exchange followed by glucocorticoid pulse therapy, the patient recovered. 1. Introduction Systemic lupus erythematosus (SLE) is an autoimmune disease with multiple organ involvement and is a common cause of secondary antiphospholipid syndrome (APS). APS is defined by arterial or venous thrombus, recurrent fetal loss, and thrombocytopenia with positive antibodies including lupus anticoagulant (LA), anticardiolipin antibodies (aCL), and antibodies to β2-glycoprotein-I (anti-β2GPI) [1, 2]. Thrombus-induced various organ infarctions such as deep vein thrombosis, stroke, pulmonary embolism, bowel, or heart ischemia, which are common in SLE patients with secondary APS. Here, we report a patient with SLE and secondary APS with coexisting renal infarction and a large thrombus over the portal vein and the inferior cava. 2. Case Report In January 2006, a 33-year-old woman was diagnosed with SLE, based on malar rash, positive ANA (1？:？640, mixed pattern), high titer of anti-dsDNA (140？IU/mL, normal <10), and autoimmune hemolytic anemia. Since then, she received immunosuppressive medications including prednisolone, azathiopurine, and hydroxychloroquine. In January 2007, splenectomy was performed on account of refractory hemolytic anemia and thrombocytopenia. In June 2007, severe nephritic syndrome with urine daily protein loss (DPL) 8？g developed. She received renal biopsy, and the biopsy revealed membranous glomerulonephritis. Monthly pulses of cyclophosphamide combined with pulse corticosteroids therapy was initiated thereafter; however, the response was poor. Persistent proteinuria (urine DPL: 5 to 10？g) was still found. In November 2007, she presented with intermittent abdominal pain in the emergency room. Physical examination revealed decreased bowel sound, positive shifting dullness, rebounding tenderness in the right lower quadrant, left costovertebral-angle tenderness, and peripheral bilateral leg edema. Laboratory data revealed the following