Anticytoplasmic antibodies in Wegener's granulomatosis.
نویسنده
چکیده
Wegener 's granulomatos is , a d isease of unknown et io logy, is character ized by a granulomatous vascul i t is of the upper and lower r e s p i r a t o r y t r a c t s . Va r y i n g d e g r e e s o f s y s t e m i c v a s c u l i t i s o f s m a l l arter ies and veins may also be seen <1>. Renal disease in the form of f o c a l o r d i f f u s e n e c r o t i z i n g g l o m e r u l i t i s i s o f t e n p r e s e n t . U n t i l recen t l y, t he d i sease was un i fo rm ly fa ta l . Today, Immunosuppress ive and cytoxic therapy are used with up to 90% of patients responding <2).
منابع مشابه
Human endothelial cells express proteinase 3, the target antigen of anticytoplasmic antibodies in Wegener's granulomatosis.
Autoantibodies directed against cytoplasmic antigens of neutrophils (ANCA), especially proteinase 3 (PR-3), have proved to be a useful clinical tool confirming the diagnosis or monitoring disease activity of Wegener's granulomatosis (WG). Although several concepts concerning the pathophysiologic potentials of ANCA have been discussed, only sparse data about ANCA-endothelium interactions have be...
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Wegener's granulomatosis is a systemic vasculitis of unknown etiology, primarily affecting the upper and lower respiratory tract and the kidneys, although there is a form restricted to the lungs. Wegener's granulomatosis occurs infrequently, with an estimated annual incidence of 8.5 cases per million. The principal symptoms at onset usually involve the upper and lower airway. The central nervou...
متن کاملAntibodies to neutrophil cytoplasmic antigens in Wegener's granulomatosis and other conditions.
The use of serum antibodies to neutrophil cytoplasmic antigens (ANCA) as a diagnostic marker for Wegener's granulomatosis and other forms of vasculitis has been assessed. Although ANCA have been described by several groups the precise antigenic targets are unknown, and detection of ANCA still relies on an indirect immunofluorescence assay technique. Several different patterns of fluorescence ha...
متن کاملWegener's granulomatosis autoantigen is a novel neutrophil serine proteinase.
Circulating IgG autoantibodies that produce cytoplasmic immunofluorescence staining of ethanol-fixed normal neutrophils have recently been found in a large percentage of patients with active Wegener's granulomatosis. Such autoantibodies are rarely found in other diseases and are therefore virtually diagnostic of Wegener's granulomatosis. The nature of the neutrophil antigen defined by these aut...
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BACKGROUND Anti-neutrophil cytoplasmic antibodies (ANCA) is autoantibodies characteristic of vasculitis diseases. A connection between ANCA and Wegener's granulomatosis was well established. The interaction of both ANCA phenotypes (PR3-ANCA and MPO-ANCA) with leukocytes provoked cell activation, which might be involved in the pathogenesis of ANCA-related Wegener's granulomatosis. METHODS In t...
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ورودعنوان ژورنال:
- Lancet
دوره 1 8441 شماره
صفحات -
تاریخ انتشار 1985