An Unusual Presentation of Myeloperoxidase-Associated Glomerulonephritis and Suspected IgA-Mediated Anti-Glomerular Basement Membrane Disease: A Case Report.

INTRODUCTION

Anti-glomerular basement membrane (GBM) disease is a rare cause of glomerulonephritis usually mediated by IgG antibodies and is associated with ANCA-associated glomerulonephritis in up to 50% of cases. IgA-mediated anti-GBM disease is extremely rare and presents diagnostic difficulties as circulating IgA antibodies will not be detected by standard serological tests for anti-GBM disease.

CASE PRESENTATION

We present the case of a 67-year-old man with rapidly progressive glomerulonephritis requiring haemodialysis at presentation. Serological testing was positive for anti-myeloperoxidase and negative for IgG anti-GBM antibodies. Kidney biopsy revealed necrotizing crescentic glomerulonephritis with linear staining of IgA along the GBM. He was treated with a combination of immunosuppression and plasma exchange and was able to become dialysis-independent.

CONCLUSION

To our knowledge, this is the first documented "double-positive" IgA anti-GBM disease and ANCA-associated glomerulonephritis.