Mesenteric revascularisation in a young patient with antiphospholipid syndrome and fibromuscular dysplasia: report of a case and review of the literature.

Abstract

Fibromuscular dysplasia or fibromuscular hyperplasia is a rare non-atherosclerotic and non-inflammatory vascular disease that primarily involves medium-size and small arteries, most commonly the renal and carotid arteries, and less frequently the vertebral, iliac, subclavian or visceral arteries (mesenteric, hepatic, splenic). Antiphospholipid syndrome is one of the most commonly acquired hypercoagulable states, defined by the association of laboratory evidence of anti-phospholipid antibodies with arterial or venous thrombosis or recurrent pregnancy losses. The presence of these antibodies is associated with an increased risk of thromboembolic phenomena, including peripheral thrombophlebitis, pulmonary thromboembolism, stroke, retinal artery occlusion, myocardial infarction, placental thrombosis and Budd-Chiari syndrome. In this report we discuss the uncommon case of a young male patient with both antiphospholipid syndrome and fibromuscular dysplasia that came to our attention for pulmonary embolism and "angina abdominis" due to occlusion of three mesenteric vessels. The possible relationship between antiphospholipid syndrome and fibromuscular dysplasia encountered in our patient still remains unclear. We treated the patient as if he had the two different diseases. After partial failure of endovascular surgery, the patient underwent surgery with reimplantation of three visceral arteries to the aorta. Subsequently he was treated with stent placement after development of a re-stenosis of one of the three reimplanted visceral arteries. The patient was treated conservatively for antiphospholipid syndrome with anticoagulant oral therapy for life.