Severe symptomatic stenosis of visceral and renal arteries leading primary antiphospholipid syndrome diagnosis.

Antiphospholipid syndrome (APS) is an autoimmune disorder with combination of at least 1 clinical and 1 laboratory criterion as defined by the SAPPORO statement. Clinical criteria result from vascular thrombosis that can affect artery, venous, or small vessel in any tissue or organ. Arterial stenosis is a rare lesion involved in APS, affecting mainly renal or intracranial arteries. We reported a case of a 33-year-old woman with abdominal angina and high blood pressure (BP). Imaging showed tight, not calcified, and hypodense stenosis of mesenteric superior artery and left renal artery, and a thrombosis of the celiac trunk. Treatment was digestive rest followed by angioplasty and stenting of mesenteric and renal artery, anticoagulation, antiplatelet, and statin therapy. Normal BP and digestive function were obtained postoperatively. Biological tests showed a positive lupus anticoagulant at diagnosis and at 12 weeks, which allowed us to make the diagnosis of APS. Physiopathology of stenosis in APS remains unclear but suggests arterial wall partial thrombosis, accelerated atherosclerosis, and/or proliferation of smooth muscle cells. We recommend screening of arterial stenosis in patients with APS and arterial symptoms, and inversely, searching for APS in young patients with atypical arterial stenosis to allow optimal therapy.