Immune-complex-mediated glomerulonephritis and pulmonary hemorrhage simulating Goodpasture syndrome.
Two female children whose clinical presentations and renal light-microscopic findings were consistent with Goodpasture syndrome are described. Immunopathologic studies demonstrated granular deposition of immunoglobulins and complement, suggesting that the renal disease was mediated by circulating immune complexes and not by anti-glomerular basement membrane antibody. Anti-GBM antibody was absent in both patients. These patients represent the first report in children of idiopathic nephritis due to immune complexes with associated pulmonary hemorrhage. The findings raise some doubt as to the accuracy of previous reports of Goodpasture syndrome in children, and also demonstrate the diagnostic and therapeutic importance of evaluation the renal immunopathology in the child with nephritis and pulmonary hemorrhage.
D019867:Anti-Glomerular Basement Membrane Disease / Q000175:diagnosis*; D001323:Autoantibodies / Q000302:isolation & purification; D001485:Basement Membrane / Q000276:immunology; D002648:Child; D003176:Complement C3 / Q000032:analysis; D003937:Diagnosis, Differential; D005260:Female; D005921:Glomerulonephritis / Q000175:diagnosis*; D006469:Hemoptysis / Q000175:diagnosis*; D006801:Humans; D007105:Immune Complex Diseases / Q000175:diagnosis*; D007136:Immunoglobulins / Q000032:analysis; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology
D001323:Autoantibodies; D003176:Complement C3; D007136:Immunoglobulins