A 25-Year Institutional Experience with Anti-Glomerular Basement Membrane Disease in Pediatric Renal Biopsy and Autopsy Specimens
SR Williamson, SP Andreoli, C Nailescu, CL Phillips. Indiana University, Indianapolis; Riley Hospital for Children, Indianapolis
Background: Anti-glomerular basement membrane (anti-GBM) disease is characterized by rapidly progressive glomerulonephritis (RPGN) and autoantibody formation against type IV collagen in the GBM. As antibodies may react with alveolar basement membrane, pulmonary hemorrhage is the third component in Goodpasture Syndrome. Exceedingly rare in children, mean age of onset is 20-30 years with a second peak at 50-70. In adults, the pulmonary component is associated with cigarette smoking and inhaled hydrocarbon vapor, although the mechanism is unclear.
Design: We reviewed renal biopsy and autopsy specimens over 25 years from a large US children's hospital, basing the diagnosis of anti-GBM disease on clinical RPGN, crescentic glomerulonephritis by light microscopy, and linear immunofluorescent (IF) IgG staining of the GBM in patients 18 or younger, correlating with serum findings.
Results: We identified 3 cases by renal biopsy and 1 at autopsy.
|Gender||Age||IgG IF||Serum anti-GBM||ANCA||Pulm. Hemorrhage|