Vasculitides C. The antineutrophil cytoplasmic antibody-associated vasculitides: Wegener's granulomatosis, microscopic polyangiitis, and the Churg-Strauss syndrome

Abstract

Many patients with Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), or the Churg-Strauss syndrome (CSS) have antineutrophil cytoplasmic antibodies (ANCA) within their serum. As a result, these three disorders are termed the ANCA-associated vasculitides (AAV), even though not all patients with these conditions have ANCA. Multiple antibodies may lead to positive immunofluorescence testing for ANCA in either perinuclear (PANCA) or cytoplasmic (C-ANCA) patterns. However, only antibodies to myeloperoxidase (MPO) and proteinase-3 (PR3) are associated with the AAV. Wegener's granulomatosis may be associated with destructive upper respiratory tract disease, including saddle-nose deformity, erosive sinusitis, and subglottic stenosis. The CSS is often associated with allergic rhinitis, nasal polyposis, or sinusitis, but is rarely associated with destructive lesions. A host of ocular lesions may occur in the AAV, including episcleritis, scleritis, peripheral ulcerative keratitis, and orbital pseudotumor. Lung disease is common in the AAV and ranges from asthma (in CSS) to nodular lesions with a tendency to cavitate (in WG) to interstitial lung disease (MPA) to alveolar hemorrhage (all forms of AAV). Segmental, necrotizing glomerulonephritis commonly accompanies the AAV, particularly WG and MPA. Eosinophilia is the sine qua non of CSS. © 2008 Springer Science+Business Media, LLC. © 2008 Springer-Verlag New York.