Autoantibodies against myeloid lysosomal enzymes in crescentic glomerulonephritis

• Published in: Kidney international Vol. 37; no. 2; pp. 799 - 806
• Main Authors: Tervaert, J. W. Cohen, Goldschmeding, R., Elema, J.D., van der Giessen, M., Huitema, M.G., van der Hem, G.K., The, T.H., von dem Borne, A.E.G.Kr, Kallenberg, C.G.M.
• Format: Journal Article
• Language: English
• Published: New York, NY Elsevier Inc 01.02.1990; Nature Publishing
• Subjects: Autoantibodies - analysis; Autoantibodies - immunology; Biological and medical sciences; Enzyme-Linked Immunosorbent Assay; Female; Fluorescent Antibody Technique; Glomerulonephritis; Glomerulonephritis - enzymology; Glomerulonephritis - immunology; Granulomatosis with Polyangiitis - enzymology; Granulomatosis with Polyangiitis - immunology; Humans; Male; Medical sciences; Middle Aged; Nephrology. Urinary tract diseases; Nephropathies. Renovascular diseases. Renal failure; Pancreatic Elastase - immunology; Peroxidase - immunology; Human; Glomerulonephritis; Urinary system disease; Enzyme; Immunological investigation; Evolutivity; Lysosome; Autoantibody; Elastase; Peroxidase
• ISSN: 0085-2538; 1523-1755
• DOI: 10.1038/ki.1990.48

Autoantibodies against myeloid lysosomal enzymes in crescentic glomerulonephritis. To investigate the possible association of crescentic glomerulonephritis (CGN) with autoantibodies to myeloid lysosomal enzymes, we tested sera from 35 consecutive patients with CGN without diagnostic immunohistological findings in their renal biopsy for the presence ofantineutrophil cytoplasmic antibodies directed against a 29 kD antigen from azurophilic granules (29 kD-ANCA), against myeloperoxidase (MPO-ANCA) and against elastase (elastase-ANCA), using antigen-catching ELISAs with well-defined monoclonal antibodies. 29 kD-ANCA were present in the sera of all nine patients with CGN as part of biopsy-proven Wegener's granulomatosis (WG), of ten patients with CGN and clinically suspected WG, and of two patients with idiopathic CGN. Sera from the remaining patients with clinically suspected WG (N = 5) or idiopathic CGN (N = 6) were negative for 29 kD-ANCA, but invariably positive for MPO-ANCA. Neither of these antibodies could be detected in sera from patients with CGN of infectious origin (N = 3), different forms of CGN (N = 7), other renal lesions (N = 34), or normal controls (N = 52). None of the sera tested were positive for elastase-ANCA. Our results indicate that both vascu-litis-associated CGN and idiopathic CGN are associated with autoantibodies against myeloid lysosomal enzymes. This finding places these disorders within one spectrum of diseases.