Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis
Joint Authors
Syed, Rafeel
Rehman, Amina
Valecha, Gautam
El-Sayegh, Suzanne
Source
Issue
Vol. 2015, Issue 2015 (31 Dec. 2015), pp.1-8, 8 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2015-11-25
Country of Publication
Egypt
No. of Pages
8
Main Subjects
Abstract EN
Rapidly progressive glomerulonephritis (RPGN) is a syndrome signified by a precipitous loss of renal function, with features of glomerulonephritis including dysmorphic erythrocyturia and glomerular proteinuria.
RPGN is associated with extensive crescent formation, and, thus, the clinical term RPGN is often used interchangeably with the pathologic term crescentic glomerulonephritis (CGN).
From an immunopathologic standpoint, primary RPGN is divided into pauci-immune GN (PICG), anti-GBM GN, and immune complex GN.
PICG, the most common etiology of primary RPGN, refers to a necrotizing glomerulonephritis with few or no immune deposits by immunofluorescence (IF) or electron microscopy (EM).
In most patients, pauci-immune CGN is a component of a systemic small vessel vasculitis such as granulomatosis with polyangiitis (GPA).
Approximately 90% of patients with PICG have circulating ANCA antibodies, leading to the nomenclature ANCA-associated vasculitis (AAV).
Recent research has identified several other antibodies associated with PICG, which is now understood to be a complex spectrum of disease with considerable overlap in terms of clinical phenotype and outcomes.
In addition, several genetic and environmental factors have recently been implicated in the pathogenesis of this disorder.
With new prognostic classifications, enhanced understanding of immunopathologic mechanisms, and novel treatment paradigms, clinical and experimental interest in PICG remains high.
American Psychological Association (APA)
Syed, Rafeel& Rehman, Amina& Valecha, Gautam& El-Sayegh, Suzanne. 2015. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis. BioMed Research International،Vol. 2015, no. 2015, pp.1-8.
https://search.emarefa.net/detail/BIM-1055343
Modern Language Association (MLA)
Syed, Rafeel…[et al.]. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis. BioMed Research International No. 2015 (2015), pp.1-8.
https://search.emarefa.net/detail/BIM-1055343
American Medical Association (AMA)
Syed, Rafeel& Rehman, Amina& Valecha, Gautam& El-Sayegh, Suzanne. Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis. BioMed Research International. 2015. Vol. 2015, no. 2015, pp.1-8.
https://search.emarefa.net/detail/BIM-1055343
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1055343