Histopathologic classification of ANCA-associated glomerulonephritis.
Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is the most common cause of rapidly progressive glomerulonephritis worldwide, and the renal biopsy is the gold standard for establishing the diagnosis. Although the prognostic value of the renal biopsy in ANCA-associated glomerulonephritis is widely recognized, there is no consensus regarding its pathologic classification. We present here such a pathologic classification developed by an international working group of renal pathologists. Our classification proposes four general categories of lesions: Focal, crescentic, mixed, and sclerotic. To determine whether these lesions have predictive value for renal outcome, we performed a validation study on 100 biopsies from patients with clinically and histologically confirmed ANCA-associated glomerulonephritis. Two independent pathologists, blinded to patient data, scored all biopsies according to a standardized protocol. Results show that the proposed classification system is of prognostic value for 1- and 5-year renal outcomes. We believe this pathologic classification will aid in the prognostication of patients at the time of diagnosis and facilitate uniform reporting between centers. This classification at some point might also provide means to guide therapy.
Berden
Annelies E
AE
Department of Pathology, Leiden University Medical Center, Leiden, Netherlands. a.e.berden@lumc.nl
Ferrario
Franco
F
Hagen
E Christiaan
EC
Jayne
David R
DR
Jennette
J Charles
JC
Joh
Kensuke
K
Neumann
Irmgard
I
Noël
Laure-Hélène
LH
Pusey
Charles D
CD
Waldherr
Rüdiger
R
Bruijn
Jan A
JA
Bajema
Ingeborg M
IM
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D001706:Biopsy; D005260:Female; D005921:Glomerulonephritis / Q000145:classification / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007678:Kidney Glomerulus / Q000473:pathology*; D007684:Kidney Tubules / Q000473:pathology; D008297:Male; D008875:Middle Aged; D055815:Young Adult
D019268:Antibodies, Antineutrophil Cytoplasmic