Fast and Furious: IgAN with Rapidly Progressive Glomerulonephritis

Introduction: IgA Nephropathy (IgAN) is the most common cause of glomerulonephritis and can manifest with a wide range of kidney impairment, ranging from asymptomatic hematuria to Rapidly Progressive Glomerulonephritis (RPGN). This later entity is uncommon, encompassing <10% of IgAN presentations, and generally leads to poor renal outcomes. Despite the many recent advances in the field of IgAN management, the optimal management of IgAN with RPGN remains unclear and current guidelines recommend treating these patients with cyclophosphamide and glucocorticoids similar to patients with renal ANCA-associated vasculitis. Case Description: A 21-year-old man presented after a month of fevers, chills, pharyngitis, non-productive cough, edema, and lower extremity palpable purpura and was found to have AKI with nephrotic range proteinuria and hematuria. History was notable for an AKI with hematuria and proteinuria shortly after an episode of pharyngitis two years earlier, though repeat labs found normal renal function with bland urine soon after and he was later lost to follow up. On his current presentation serologic testing and renal imaging was unremarkable. Renal biopsy was obtained which showed findings of a crescentic glomerulonephritis with IgA dominant deposits. He received treatment with pulse dose steroids and cyclophosphamide. He required hemodialysis due to hypervolemia and hyperkalemia, and unfortunately has remained dialysis-dependent four months out from his presentation. Discussion: This case of IgAN with RPGN demonstrates the rapid development of kidney failure in a young patient despite prompt and intensive immunosuppression. The current recommendations for treatment of IgAN with RPGN are based on limited prospective data as there are no randomized controlled trials studying this entity. Ongoing research is needed to better inform management of this uncommon but devastating IgAN presentation.