Elsevier

Neurologic Clinics

Volume 15, Issue 4, 1 November 1997, Pages 887-891
Neurologic Clinics

WEGENER'S GRANULOMATOSIS AND ANCA SYNDROMES

https://doi.org/10.1016/S0733-8619(05)70353-4Get rights and content

Wegener's granulomatosis (WG) is a form of necrotizing vasculitis that differs from other forms of systemic vasculitis in its prediliction for involvement of the upper and lower respiratory tracts and the kidneys.21 The diagnosis of WG is based on the criteria of the American College of Rheumatology (ACR) published in 1990:10 Nasal or oral inflammation with painful or painless oral ulcers or purulent bloody nasal discharge; abnormal chest radiography showing the presence of nodules, fixed infiltrates, or cavities; urinary sediment with microhematuria (>5 RBCs per high power field) or red cell casts in urinary sediment; and granulomatous inflammation on biopsy with histologic changes showing granulomatous inflammation within the wall of an artery or in the perivascular or extra vascular area (artery or arteriole). A patient who meets at least two of these four criteria may be diagnosed as having classic WG. An earlier and stricter classification required that the modifier necrotizing be added to the biopsy features, but this is generally no longer adhered to.

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ANTINEUTROPHIL CYTOPLASMIC AUTOANTIBODIES

In 1985, the presence of autoantibodies to components of granulocytes was first described in relation to WG and were referred to as antineutrophil cytoplasmic autoantibodies (ANCA).19 Prior to these observations, neither WG nor any of the other systemic vasculitides had a serologic marker to aid in diagnosis, analagous to antinuclear antibodies or rheumatoid factors found in other collagen vascular diseases. Using indirect immunofluorescence microscopy with alcohol fixed human neutrophiles as a

ANTINEUTROPHL CYTOPLASMIC AUTOANTIBODY–POSITIVE DISEASE AND THE NERVOUS SYSTEM

Both the central and peripheral nervous system are involved in WG and the other clinically related, biopsy proven, and often ANCA-positive vasculitides. Unlike the Churg-Strauss syndrome, where nervous system involvement is one of the major ACR criteria for diagnosis,12 it is much less frequent in WG and is not one of the diagnostic criteria.10

In 1958, Walton identified neurologic features in 35 of 56 reported cases of WG, with peripheral neuropathy the most constant finding.20 Drachman

SUMMARY

Classical WG involves the nervous system in 25% to 30% of patients in recently reported series. The discovery of ANCA helped to unify WG and its variants into a related spectrum of diseases, although biopsy remains the definitive diagnostic test. Modern treatment has markedly altered the outcome in WG and ANCA-related variants, and this treatment regimen has been applied to other types of vasculitis, often with similar favorable results. Newer imaging studies with greater sensitivity,

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*

From the Department of Medicine, College of Physicians and Surgeons, Columbia University, New York, New York

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Copyright © 1997 W. B. Saunders Company. Published by Elsevier Inc. All rights reserved.