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Recent Advances in Chest Medicine |

Antineutrophil Cytoplasmic Antibody-Associated Vasculitides and Respiratory Disease

Jose A. Gómez-Puerta, MD, PhD; José Hernández-Rodríguez, MD; Alfonso López-Soto, MD, PhD; Xavier Bosch, MD, PhD
Author and Funding Information

Affiliations: From the Departments of Rheumatology (Dr. Gómez-Puerta), Autoimmune and Systemic Diseases (Dr. Hernández-Rodríguez), and Internal Medicine (Drs. López-Soto and Bosch), Hospital Clínic, Institut d'Investigacions Biomèdiques August Pí i Sunyer (Idibaps), University of Barcelona, Barcelona, Spain.

Correspondence to: Xavier Bosch, MD, PhD, Internal Medicine Department, Hospital Clínic, Villarroel 170, 08036, Barcelona, Spain; e-mail: xavbosch@clinic.ub.es


This study was in part supported by the Ministerio de Educación y Ciencia and Fondo Europeo de Desarrollo Regional (SAF 08/04328) and Marató TV3 (06/0710).

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (www.chestjournal.org/site/misc/reprints.xhtml).


© 2009 American College of Chest Physicians


Chest. 2009;136(4):1101-1111. doi:10.1378/chest.08-3043
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Vasculitides associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCAs) are a well-established subgroup affecting small- to medium-sized vessels that are commonly recognized as ANCA-associated vasculitis, which includes necrotizing granulomatous vasculitis (NGV) [formerly Wegener granulomatosis], microscopic polyangiitis (MPA), and Churg-Strauss syndrome. NGV usually starts as a granulomatous disease of the respiratory tract and progresses to systemic disease with proteinase 3 (PR3)-ANCA-associated vasculitis, suggesting an aberrant cell-mediated immune response to exogenous or endogenous antigens in the respiratory tract and resulting in granuloma formation. In NGV, granulomata may represent lymphoid structures ultimately responsible for PR3-ANCA production. In both NGV and MPA, necrotizing glomerulonephritis and necrotizing pulmonary capillaritis may well result from an injury orchestrated by ANCA. Untreated NGV and MPA normally are rapidly progressive and fatal. Pulmonary capillaritis with alveolar hemorrhage is a severe complication in patients with MPA and NGV. Because plasma exchange removes circulating ANCAs and other proteins from the blood, its use has been advocated in critical situations of severe renal and pulmonary involvement. However, no studies of plasma exchange in ANCA-associated vasculitis focused on pulmonary involvement have been reported. Dissecting the mechanisms of inflammation may identify molecular targets for future therapies in ANCA-associated vasculitis. Thus, biological agents are emerging as potential therapies in refractory cases. Notably, rituximab and infliximab have been trialed with apparent initial clinical success.

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