Antiphospholipid Antibodies and Vasculitis

Abstract

This chapter focuses on the relationship between antiphospholipid syndrome (APS) and vasculities. The vascular lesions occurring with the APS, which form the basis of the clinical syndrome, are associated with a non-inflammatory vasculopathy resulting in recurrent venous and arterial thrombosis, fetal losses as well as livedo reticularis, valvular heart disease, and thrombocytopenia. Vasculitis in association with aPL, either anticardiolipin antibody (aCL) or lupus anticoagulant (LA), has been encountered in various circumstances, mainly when patients suffering from systemic lupus erythematosus (SLE) have vasculitic lesions associated primarily with the SLE itself in which the vasculitis occurs due to the underlying disease. Several varieties of skin lesions have been documented in patients with the APS, which include livedo reticularis, livedoid vasculitis, thrombophlebitis, cutaneous infarctions and gangrene of digits, ulcerations, lesions resembling vasculitis (nodules, macules), cutaneous necrosis, subungual splinter hemorrhages, and, less commonly, discoid lupus and Degos' disease. Several case reports documenting the association of polyarteritis nodosa (PAN) with aPL have been published. Pradeiro et al. reported a 63-years old woman with LA who developed digital necrosis of her left foot. Pathology of the two amputated digits revealed destruction of the vessel walls of the majority of the small and medium-sized arteries by a dense polymorphonuclear infiltrate.