Nephropathy associated with sickle cell anemia: an autologous immune complex nephritis. I. Studies on nature of glomerular-bound antibody and antigen identification in a patient with sickle cell disease and immune deposit glomerulonephritis.
The nature of the glomerular-bound antibody and the putative antigen was investigated in one of the patients with sickle cell disease and immune deposit membranoproliferative glomerulonephritis by immunohistologic and glomerular antibody elution. Renal proximal tubular epithelial antigen was localized in association with immunoglobulins G (IgG), M (IgM), Clq fraction of the first component of complement (Clq) and the third component of complement (C3) in a granular pattern along the glomerular basement membrane of the patient's kidney. IgG and IgM were eluted from glomeruli. These immunoglobulins fixed to the proximal tubules of normal human kidney by direct immunofluorescence. This localization was abolished by absorption of the eluted immunoglobulins with renal tubular epithelial (RTE) antigen. The IgG eluted from the glomeruli blocked the fixation of rabbit anti-RTE antigen to normal proximal tubular brush border. These studies suggest that the nephritis in this patient was due to deposition of complexes or RTE antigen and specific antibody. An autologous immune complex nephritis may develop in some patients with sickle cell anemia secondary to RTE antigen released possibly after renal ischemia or some other phenomenon causing renal tubular damage.
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000276:immunology; D000818:Animals; D000936:Antigen-Antibody Complex; D000941:Antigens; D000942:Antigens, Bacterial; D001666:Binding Sites, Antibody; D002118:Calcium; D003165:Complement System Proteins; D004848:Epithelium / Q000276:immunology; D005260:Female; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000209:etiology* / Q000276:immunology; D006041:Goats / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007678:Kidney Glomerulus / Q000276:immunology*; D007684:Kidney Tubules / Q000276:immunology; D007687:Kidney Tubules, Proximal / Q000276:immunology; D011817:Rabbits / Q000276:immunology; D013194:Staining and Labeling; D013291:Streptococcus / Q000276:immunology
D000936:Antigen-Antibody Complex; D000941:Antigens; D000942:Antigens, Bacterial; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D003165:Complement System Proteins; D002118:Calcium