Case Report

Polyarteritis Nodosa Complicated by Antiphospholipid Syndrome

Authors: Jorge L. Musuruana, MD, Javier A. Cavallasca, MD

Abstract

Polyarteritis nodosa is a necrotizing vasculitis of small and medium-sized arteries that spares the smallest blood vessels (arterioles, venules, and capillaries). Antiphospholipid syndrome is an autoimmune disorder characterized by venous or arterial thrombosis and/or by fetal losses, associated with antiphospholipid antibodies. The association of both diseases is infrequent. This case report discusses a male patient with a diagnosis of polyarteritis nodosa who, after 7 years of being diagnosed with vasculitis, showed ischemic lesions in his legs associated with high titers of anticardiolipin antibodies, along with angiographic and histologic evidence of thrombosis. Despite immunosuppressive and anticoagulant therapy, his lesions progressed, and both legs had to be amputated.


Key Points


* The association of polyarteritis nodosa and antiphospholipid syndrome is uncommon.


* Patients with polyarteritis nodosa may, even several years after the onset of vasculitis symptoms, develop clinical and serological signs of antiphospholipid syndrome.


* The association of both diseases represents a diagnostic and therapeutic challenge, because despite receiving an adequate immunosuppressor and anticoagulant treatment, the clinical course may be very aggressive.

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