A severe renal bleeding as a complication of coumarin therapy in Behçet's disease

Abstract

Behçet's disease is a systemic inflammatory disease, which predisposes patients to venous or arterial thrombosis. We report a case of 41-year-old patient who presented for several years with recurrent fevers, arthralgias, episodes of skin eruptions and recurrent bilateral deep venous ileofemoral thromboses, extending into the inferior vena cava, despite the oral anticoagulant therapy. Additionally, he also reported recurrent aphthous oral and genital lesions, and eye problems. A laboratory picture of chronic inflammation, normocytic anaemia and a finding of lupus anticoagulant/antiphospholipid antibodies were observed. PET raised a high suspicion of vasculitic PTT-lupus anticoagulant process, involving the large vessels. During an anticoagulant therapy by warfarin (dose in upper limit of therapeutic range), the left kidney had to be removed because of acute retroperitoneal haemorrhage. On histologic examination, the picture of nephritis was described. A detailed retrospective reevaluation of complex history as well as other clinical findings strongly raised the suspicion of a systemic vasculitic syndrome, such as Behçet's disease, accompanied by antiphospholipid syndrome. The patient responded well to the combination of colchicine and anticoagulant therapy by low-molecular-weight heparin. The episodes of fever, skin eruptions and other symptoms disappeared. We assume that this is a case of an unusual course of Behçet's disease presenting with a rare kidney involvement. Kidney disorder complicated the oral anticoagulant therapy for recurrent venous thromboses and led to the excessive renal bleeding, requiring nephrectomy. Recognition that nephritis is associated with Behçet's disease may be useful to prevent severe renal bleeding in relation to anticoagulant therapy.

Spontaneous retroperitoneal haemorrhage as a first presentation of Behçet disease

“…There have been a few published cases of renal artery haemorrhage in patients already diagnosed with Behçet disease, but they were also on anticoagulants. 2,3 In Behçet disease venous disease usually presents as thrombosis, and arterial usually as aneurysms. 4 SRH is a rare presentation, and a meta-analysis to determine the aetiologies revealed renal neoplasm to be the commonest (61.5%), with angiomyolipoma occurring most frequently followed by renal cell carcinoma.…”