A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegeners Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM AntibodiesReportar como inadecuado




A Case of Simultaneous, Biopsy-Proven, Classic, ANCA-Positive Wegeners Granulomatosis and Anti-GBM Disease, but without Detectible Circulating Anti-GBM Antibodies - Descarga este documento en PDF. Documentación en PDF para descargar gratis. Disponible también para leer online.

TheScientificWorldJOURNAL - Volume 10 2010, Pages 1078-1083

Case Study Evanston Northwestern Healthcare, Northwestern University Feinberg School of Medicine, Chicago, USA

Received 20 January 2010; Revised 10 May 2010; Accepted 11 May 2010

Academic Editor: Ali K. Abu-Alfa

Copyright © 2010 Aleksandra Gmurczyk et al.

Abstract

Wegener-s granulomatosis WG is a systemic, necrotizing, granulomatous vasculitis of unknown etiology. Approximately 75% of cases present as classic WG with both pulmonary and renal involvement, while the remaining 25% of patients present with a limited form with either predominantly upper or lower respiratory tract symptoms. Ninety percent of WG patients have circulating anti–neutrophil cytoplasmic antibodies ANCA, and approximately 10% have both circulating ANCA antibodies and concomitant anti–glomerular basement membrane anti-GBM disease on renal biopsy. Virtually all of these patients also have circulating anti-GBM antibodies. While it has been reported that some patients with ANCA vasculitis have circulating anti-GBM antibodies, and patients with anti-GBM disease may have positive ANCA, review of the literature does not demonstrate other cases of biopsy-proven, simultaneous, ANCA-associated vasculitis and anti-GBM disease. We report a case of simultaneous, biopsy-proven, classic, ANCA-positive WG and anti-GBM disease, but without detectible circulating anti-GBM antibodies. We present findings characteristic of both WG and linear IgG deposition along the GBM suggesting concurrent anti-GBM disease, in the absence of detectable circulating anti-GBM antibodies. Possible theories to explain the absence of these antibodies are discussed.





Autor: Aleksandra Gmurczyk, Shubhada N. Ahya, Robert Goldschmidt, George Kim, L. Tammy Ho, and Kevin Nash

Fuente: https://www.hindawi.com/



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