An unusual syndrome of a devastating noninflammatory vasculopathy associated with anticardiolipin antibodies: report of two cases

Abstract

We describe 2 patients with marked elevations of anticardiolipin antibodies who developed multiple-organ disease characterized by a noninflammatory vasculopathy. Their cases were remarkable for the fulminant nature of their thrombotic diathesis, which was heralded by a prominent livedo reticularis of the extremities. Both patients had a serologic profile and salivary gland biopsy findings that were consistent with a diagnosis of primary Sjögren's syndrome.