Background and Aims

Antiphospholipid syndrome (APS) occurs in 1% of cases as catastrophic variant (CAPS), a highly lethal condition characterized by acute onset of multiple thromboses and/or microangiopathy. Aortic involvement is unusual and requires a high suspicion for diagnosis.

Methods

We describe the case of APS who developed acute aorto-iliac occlusion (Leriche syndrome) as CAPS debut. We also performed a review of the literature by Medline and EMBASE databases

Results

A 64-year-old male patient with primary APS history and previous arterial events was on treatment with only clopidogrel. He presented with aortic barrage so he underwent aorto-bifemoral by pass. At day 1 after surgery he developed thrombocytopenia, hemolytic anemia, livedo racemosa, cutaneous necrosis at lower limbs and alveolar hemorrhage. Antiphosholipid antibodies resulted positive at extremely high titer. Blood smear revealed schistocytes>6%. Skin biopsy documented thrombosis of dermal vessels. Treatment with methylprednisolone pulses, anticoagulation and plasma exchanges was started with full recovery.

Eight cases of Leriche syndrome in APS have been described so far: 5 primary forms (PAPS), 1 case associated with relapsing polychondritis. In 2 cases data were missing. In 3 cases aortic barrage represented APS onset manifestation, 4 cases were preceded by arterial thromboses, while none by venous thrombotic events. No proven evolution into CAPS was previously reported.

Conclusions

Aortic occlusion should be suspected in patients with APS and consistent clinical presentation, particularly when other cardiovascular risk factors or history of arterial thrombosis are present. CAPS is a life-threatening complication of surgery in patients with APS and must be systematically excluded in presence of symptoms.