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Nephrol Dial Transplant (2001) 16: 39-44
© 2001 European Renal Association-European Dialysis and Transplant Association

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

Marie A. P. Audrain1, Richard Sesboüé2, Thierry A. R. Baranger3, Jane Elliott4, Angelo Testa3, Jean-Pierre Martin2, C. Martin Lockwood4 and Vincent L. M. Esnault,1,3

1 Immunology Department 3 Nephrology and Clinical Immunology Department, Hôtel Dieu, Nantes, France 2 INSERM Unité 295, Faculté de Médecine-Pharmacie Rouen, France 4 University of Cambridge, School of Clinical Medicine, Cambridge, UK

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 ({alpha}Gr). 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 {alpha}Gr or antigen-specific ELISA [PR3, HLE, MPO, lactoferin (LF) and bactericidal/ permeability increasing protein (BPI)].

Results. The incidence of antibodies directed against {alpha}Gr and HLE but not PR3, MPO, LF or BPI was increased in the PiZZ as compared to the PiMM group (Fisher probability respectively P<0.0001 and P<0.05).

Conclusions. ANCA not directed against classical antigens (MPO and PR3) may be found in PiZZ patients. However, these patients do not develop systemic vasculitis features. Therefore, alpha1-antitrypsin deficiency is not sufficient to induce ANCA positive vasculitides, and may only act as a second hit amplifying factor.

Keywords: alpha1-antitrypsin; ANCA; elastase; pro-tease inhibitor; proteinase 3; systemic vasculitis

Pr VLM Esnault, Service de Néphrologie-Immunologie Clinique, Hôtel Dieu, 30 bd Jean Monnet, F-44093 Nantes, France.


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