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Association of cerebral vasculitis with a lupus anticoagulant. A case with brain pathology.

Abstract
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.
AuthorsE Toussirot, D Figarella-Branger, P Disdier, J R Harle, P J Weiller
JournalClinical rheumatology (Clin Rheumatol) Vol. 13 Issue 4 Pg. 624-7 (Dec 1994) ISSN: 0770-3198 [Print] Germany
PMID7697967 (Publication Type: Case Reports, Journal Article)
Chemical References
  • Adrenal Cortex Hormones
  • Lupus Coagulation Inhibitor
Topics
  • Adrenal Cortex Hormones (therapeutic use)
  • Aged
  • Brain Diseases (drug therapy, etiology, pathology)
  • Cerebral Cortex (pathology)
  • Fatal Outcome
  • Female
  • Humans
  • Lupus Coagulation Inhibitor (analysis)
  • Lupus Erythematosus, Systemic (complications, diagnosis)
  • Thrombocytopenia (etiology, physiopathology)
  • Vasculitis (drug therapy, etiology, pathology)

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