[Severe visceral necrotizing angiitis with hereditary immune disorders: a case]

The authors report a new case of necrosing angeitis similar to those individualised by Churg and Strauss in 1951. After a persistent rhinitis, the patient developed serious asthma, left hemiplegia 8 months later (secondary to an intracerebral hemorrhage) polyneuritis and extensive necrosis of the small intestine. There was no renal or cardiac involvement. Anatomopathological examination of the small intestine revealed an arteriolitis with periarterial granuloma and severe inflammation. Complementary investigations revealed a hypereosinophilia, an inflammatory syndrome, a reduction in the hemolytic 50 complement and of the C4 fraction. Corticosteroid therapy associated with cyclophosphamide formed the basis of treatment. Plasmapheresis have been a useful aid. The follow-up is too short to assess the long term prognosis. A familial immune deficiency, disseminated lupus erythematosis in a sister, and rheumatoid purpura in one of the patient's children are interesting features of this text.