Immunofluorescence studies of dense deposit disease. The presence of railroad tracks and mesangial rings.
Immunopathologic analysis was carried out on renal tissue from patients with membranoproliferative glomerulonephritis, nine with type I and nine with type II (dense deposit disease). A specific finding in all patients with type II, but not type I, was the presence of C3 along the margin but not within the central portion of dense deposit material in the glomerular basement membrane giving a double linear appearance (railroad tracks); C3 was also present within the mesangium, outlining numerous circular structures (mesangial rings). By phase contrast, electron microscopy, and dual label fluorescence studies, the railroad tracks and mesangial rings were shown to outline dense deposit material. Mesangial rings contained properdin (four of nine patients); railroad tracks contained properdin (five of nine patients) and C4 (four of nine patients); no other complement components or immunoglobulins were present. Studies using rhodamine-conjugated rabbit antibody to human glomerular basement membrane demonstrated no reactivity with the dense deposit material itself. In type I and to a lesser extent in type II, granular deposits of C3, C4, properdin, IgM, and IgG were present along the glomerular basement membrane, suggesting that immune complexes play a role in both diseases. Dense deposit transformation of the glomerular basement membrane may be a consequence of a highly specific injury.
D001323:Autoantibodies / Q000032:analysis; D001485:Basement Membrane / Q000276:immunology; D003172:Complement C1 / Q000032:analysis; D003175:Complement C2 / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D003181:Complement C4 / Q000032:analysis; D011415:Complement Factor B / Q000032:analysis; D003165:Complement System Proteins / Q000032:analysis*; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000276:immunology*; D006801:Humans; D007070:Immunoglobulin A / Q000032:analysis; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D007136:Immunoglobulins / Q000032:analysis*; D007668:Kidney / Q000276:immunology; D007678:Kidney Glomerulus / Q000276:immunology*; D011414:Properdin / Q000032:analysis*
D001323:Autoantibodies; D003172:Complement C1; D003175:Complement C2; D003176:Complement C3; D003181:Complement C4; D007070:Immunoglobulin A; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D007136:Immunoglobulins; D011414:Properdin; D003165:Complement System Proteins; D011415:Complement Factor B