Association of cerebral vasculitis with a lupus anticoagulant. A case with brain pathology.
The authors report the case of a sixty-five year old woman initially suffering from a thrombocytopenia. The patient was diagnosed as having an autoimmune disease with a lupus anticoagulant, positive antinuclear antibodies and negative anti-DNA antibodies. She then developed an encephalopathy which was fatal despite corticosteroids. Brain pathology revealed a vasculitis with some giant cells, evoking a granulomatous angiitis of the central nervous system. These clinical and biological features suggest a systemic lupus erythematosus with vasculitis or a primary granulomatous angiitis of the central nervous system. Taking into account the clinical manifestations and the presence of a lupus anticoagulant, we finally preferred to identify it as a primary antiphospholipid antibodies syndrome, despite absence of anticardiolipin antibodies. Contrary to thrombosis, vasculitis is rarely associated with an anticardiolipin antibody or a lupus anticoagulant. However, vasculitis in the course of primary antiphospholipid antibodies syndrome has been reported previously as in this case report.
Toussirot
E
E
Department of Internal Medicine, CHU La Timone Marseille, France.
Figarella-Branger
D
D
Disdier
P
P
Harle
J R
JR
Weiller
P J
PJ
D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D001927:Brain Diseases / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016682:Lupus Coagulation Inhibitor / Q000032:analysis*; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis; D013921:Thrombocytopenia / Q000209:etiology* / Q000503:physiopathology; D014657:Vasculitis / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology*
D000305:Adrenal Cortex Hormones; D016682:Lupus Coagulation Inhibitor