Analysis of anti‐neutrophil cytoplasmic antibodies (ANCA): frequency and specificity in a sample of 191 homozygous (PiZZ) alpha1‐antitrypsin‐deficient subjects

Background. ANCA are autoantibodies directed against polymorphonuclear cell antigens, mainly proteinase 3 (PR3) and myeloperoxidase (MPO), which are implicated in the pathogenesis of small‐vessel necrotizing vasculitis. Alpha1‐antitrypsin is the main inhibitor of neutral serine proteinase [i.e. huma...

Full description

Saved in:
Bibliographic Details
Published in:Nephrology, dialysis, transplantation dialysis, transplantation, 2001-01, Vol.16 (1), p.39-44
Main Authors: Audrain, Marie A. P., Sesboüé, Richard, Baranger, Thierry A. R., Elliott, Jane, Testa, Angelo, Martin, Jean‐Pierre, Lockwood, C. Martin, Esnault, Vincent L. M.
Format: Article
Language:English
Subjects:
Adult
Aged
alpha 1-Antitrypsin Deficiency - genetics
alpha 1-Antitrypsin Deficiency - immunology
alpha 1-Antitrypsin Deficiency - pathology
alpha1‐antitrypsin
ANCA
Antibodies, Antineutrophil Cytoplasmic - blood
Antibody Specificity
Antimicrobial Cationic Peptides
Blood Proteins - immunology
Case-Control Studies
Child
Child, Preschool
elastase
Enzyme-Linked Immunosorbent Assay
Female
Fluorescent Antibody Technique, Indirect
Homozygote
Humans
Infant
Lactoferrin - immunology
Leukocyte Elastase - immunology
Male
Membrane Proteins
Middle Aged
Myeloblastin
Peroxidase - immunology
Phenotype
proteinase 3
pro‐tease inhibitor
Serine Endopeptidases - immunology
systemic vasculitis
Vasculitis - genetics
Vasculitis - immunology
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Background. ANCA are autoantibodies directed against polymorphonuclear cell antigens, mainly proteinase 3 (PR3) and myeloperoxidase (MPO), which are implicated in the pathogenesis of small‐vessel necrotizing vasculitis. Alpha1‐antitrypsin is the main inhibitor of neutral serine proteinase [i.e. human leukocyte elastase (HLE) and PR3] present in PMN alpha‐granules (αGr). An association first reported by us between PR3 ANCA and the deficient PiZZ phenotype in ANCA‐positive systemic vasculitis, now widely confirmed by others, led us to study the incidence and specificity of ANCA among PiZZ subjects. Methods. We tested a population of 191 PiZZ (273 sera) for ANCA activity versus 272 PiMM matched control subjects using αGr or antigen‐specific ELISA [PR3, HLE, MPO, lactoferin (LF) and bactericidal/ permeability increasing protein (BPI)]. Results. The incidence of antibodies directed against αGr and HLE but not PR3, MPO, LF or BPI was increased in the PiZZ as compared to the PiMM group (Fisher probability respectively P
ISSN:0931-0509
1460-2385
DOI:10.1093/ndt/16.1.39