Wegener's Granulomatosis

Abstract

Wegener's granulomatosis is characterized by necrotizing granulomatous vasculitis. The localized form involves The upper or lower respiratory tracts, or both; in the commoner generalized form, the kidneys are also involved. Twenty-one patients with this disease were studied at the National Institutes of Health. The typical lesions in the kidney were focal and segmental glomerulonephritis. Electron microscopy showed dense deposits in some kidneys in a pattern suggesting immune complexes. Cytotoxic immunosuppressive therapy is the treatment of choice. Long-lasting remissions of many years' duration have been induced in this once universally fatal illness. Cyclophosphamide therapy led to severe lymphocytopenia and mild granulocytopenia. Profound changes in granulocyte levels were not required for an effective therapeutic response. Cyclophosphamide therapy has various in-vitro and in-vivo immunosuppressive effects, and we still do not know which of these effects is responsible for the therapeutic response.