Idiopathic rapidly progressive glomerulonephritis with C3 nephritic factor and hypocomplementemia.
A 7-year-old boy with mild renal failure and signs and symptoms of acute poststreptococcal glomerulonephritis including severe hypocomplementemia had, by renal biopsy, numerous crescents but no deposits in the glomerular capillary loops. Instead, deposits identical in location and composition to those described for children with idiopathic rapidly progressive glomerulonephritis were present. The severe hypocomplementemia was found to be due to high levels of C3 nephritic factor; niether nephritic factor nor hypocomplementemia has been reported in rapidly progressive glomerulonephritis of the idiopathic type. Following prompt therapy with methylprednisolone intravenously, serologic abnormalities disappeared and renal function greatly improved, but a later biopsy showed 50% of the glomeruli obliterated by scarring. The case is of importance not only in indicating that severe hypocomplementemia does not rule out idiopathic rapidly progressive glomerulonephritis but also in adding to the list of diseases in which nephritic factor can be found.
D000936:Antigen-Antibody Complex; D002648:Child; D003178:Complement C3 Nephritic Factor / Q000032:analysis*; D003169:Complement Inactivator Proteins / Q000032:analysis*; D003165:Complement System Proteins / Q000032:analysis*; D005455:Fluorescent Antibody Technique; D005921:Glomerulonephritis / Q000175:diagnosis / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007678:Kidney Glomerulus / Q000648:ultrastructure; D008297:Male
D000936:Antigen-Antibody Complex; D003178:Complement C3 Nephritic Factor; D003169:Complement Inactivator Proteins; D003165:Complement System Proteins