An Interesting Case of Anti-Glomerular Basement Membrane Disease Mediated By IGA Autoantibodies

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Conference Proceeding

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American Journal of Kidney Diseases


Anti-glomerular basement membrane (anti-GBM) disease is an autoimmune condition in which circulating autoantibodies target the type IV collagen of the basement membranes found in the glomeruli and/or alveoli. Patients typically present with alveolar hemorrhage and rapidly progressive glomerulonephritis (RPGN) characterized histologically by crescents and linear deposition of IgG in the GBM. We describe a case of renal-limited anti-GBM disease with IgA deposition along the GBM and crescentic glomerular disease. The patient is a 75 year old male with a history of CKDIII who presented with dyspnea and leg edema over 1 month. His serum creatinine on admission was 3.24mg/dL, up from his baseline of ~1.8 two months ago. Urine chemistry revealed RBC>20, and nephrotic range proteinuria (16.02 g/d), . Immunologic studies were negative for anti-GBM IgG antibodies, ANA, ANCA and phospholipid receptor A2 IgG antibodies. Complement C3 and C4 levels were normal. Protein electrophoresis did not reveal monoclonal proteins. Ultrasound examination revealed ~13cm kidneys with normal corticomedullary differentiation. CT-chest was not consistent with alveolar hemorrhage. The renal biopsy revealed glomerulonephritis with cellular crescents in the background of moderate to severe interstitial fibrosis and tubular atrophy. A total of 22 glomeruli were seen, 2 of which showed cellular crescents and 10 of which showed fibrocellular crescents. Immunofluorescence of 8 glomeruli revealed diffuse linear glomerular basement membrane staining for IgA (3+), kappa (2+) and lambda (3+) light chains. A positive segmental granular-linear GBM staining is seen with IgG (1+) and C3( 2+). The other stains (IgM and C1q) are negative in glomeruli. The patient was started on cyclophosphamide and prednisone after 3 doses of methylprednisolone. He additionally received 7 sessions of plasmapheresis in an attempt to prevent progression to ESRD. Upon discharge creatinine was 4.69mg/dL and GFR was 13 , and he was ultimately started on hemodyalisis. In summary, anti-GBM disease mediated by IgA is incredibly rare, and cannot be screened for by routine ELISA designed to detect circulating IgG autoantibodies. Diagnosis relies on kidney biopsy and histopathology.




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National Kidney Foundation Spring Clinical Meeting, April 11-15, 2023, Austin, TX.

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