[1547] 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.

GenderAgeIgG IFSerum anti-GBMANCAPulm. Hemorrhage

Two patients presented with pulmonary complaints and had pulmonary hemorrhage during admission, neither with known inhaled exposure. Remote hemoptysis with smoking was present for patient 3, while a fourth had exposure to engine exhaust and renal symptoms only. Interestingly, the autopsy case (1) showed + IgG IF despite undetectable serum anti-GBM level and + serum ANCA (Fig 1). The 3 surviving patients continue to do well (6 months - 18 years later), one after successful renal transplant, one re-listed for transplant failure, and one on peritoneal dialysis.

Conclusions: Although anti-GBM remains rare in children, the US Renal Data System suggests that more cases occur than previously reported. Our sample size is small, but the association with inhaled agents may exist for children as well as adults. Serum anti-GBM antibody is typically present, but adult cases with undetectable levels have been recently described. Some patients are both anti-GBM and ANCA +, with a small subset ANCA +, anti-GBM -. To our knowledge, ours is the first such described pediatric case.
Category: Kidney (does not include tumors)

Monday, March 22, 2010 9:30 AM

Poster Session I Stowell-Orbison/Surgical Pathology/Autopsy Awards Poster Session # 207, Monday Morning


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