Antineutrophil cytoplasmic antibody negative pauci-immune extracapillary glomerulonephritis

Aim Pauci‐immune extracapillary glomerulonephritis (PEGN) is one of the most common causes of rapidly progressive glomerulonephritis and is usually associated with circulating anti‐neutrophil cytoplasmic antibodies (ANCAs). However, a significant number of individuals with PEGN test negative for ANC...

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Bibliographic Details
Published in:Nephrology (Carlton, Vic.) Vic.), 2016-04, Vol.21 (4), p.301-307
Main Authors: Villacorta, Javier, Diaz-Crespo, Francisco, Acevedo, Mercedes, Guerrero, Carmen, Mollejo, Manuela, Fernandez-Juarez, Gema
Format: Article
Language:English
Subjects:
Adult
Aged
Antibodies, Antineutrophil Cytoplasmic - blood
Antibodies, Antineutrophil Cytoplasmic - immunology
antibody
Biomarkers - blood
Cell Proliferation
crescent
Disease Progression
Female
glomerulonephritis
Glomerulonephritis - diagnosis
Glomerulonephritis - immunology
Glomerulonephritis - mortality
Glomerulonephritis - therapy
Humans
Immunosuppressive Agents - therapeutic use
Kaplan-Meier Estimate
Kidney - immunology
Kidney - pathology
Male
Middle Aged
Necrosis
Plasmapheresis
Predictive Value of Tests
Proportional Hazards Models
Recurrence
Retrospective Studies
Risk Factors
Serologic Tests
Spain
Systemic Vasculitis - diagnosis
Systemic Vasculitis - immunology
Systemic Vasculitis - mortality
Systemic Vasculitis - therapy
Time Factors
Treatment Outcome
vasculitis
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Summary:Aim Pauci‐immune extracapillary glomerulonephritis (PEGN) is one of the most common causes of rapidly progressive glomerulonephritis and is usually associated with circulating anti‐neutrophil cytoplasmic antibodies (ANCAs). However, a significant number of individuals with PEGN test negative for ANCA and this study aimed to analyze the characteristics of this subgroup of patients. Methods Patients from two centres who were diagnosed with PEGN between 1997 and 2014 were studied retrospectively. Clinicopathological characteristics and renal outcome were compared between patients presenting with pauci‐immune necrotizing extracapillary glomerulonephritis associated or not with the presence of circulating ANCA. Results Among the 114 patients with PEGN, 29 (25.4%) were ANCA negative. Compared with the 85 ANCA‐positive patients, ANCA‐negative patients were younger at the onset (54.8 ± 17.2 vs 62 ± 14.0 years; P 
ISSN:1320-5358
1440-1797
DOI:10.1111/nep.12608