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2015 ; 2015
(ä): 402826
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Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis
#MMPMID26688808
Syed R
; Rehman A
; Valecha G
; El-Sayegh S
Biomed Res Int
2015[]; 2015
(ä): 402826
PMID26688808
show ga
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.