An unusual syndrome of a devastating noninflammatory vasculopathy associated with anticardiolipin antibodies: report of two cases.
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.
D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D001323:Autoantibodies / Q000032:analysis*; D001779:Blood Coagulation Factors / Q000032:analysis / Q000276:immunology; D002308:Cardiolipins / Q000276:immunology*; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000627:therapeutic use; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D016682:Lupus Coagulation Inhibitor; D008297:Male; D010956:Plasmapheresis; D013577:Syndrome; D013927:Thrombosis / Q000276:immunology* / Q000628:therapy
D000925:Anticoagulants; D001323:Autoantibodies; D001779:Blood Coagulation Factors; D002308:Cardiolipins; D016682:Lupus Coagulation Inhibitor; D003520:Cyclophosphamide