IgA Nephropathy with Diffuse, Proliferative, Sclerosing Glomerulonephritis, and Crescent Formation
This article was published in American Journal of Kidney Diseases, Volume 83, Issue 4 Supplement 2, pages S110-S111.
The published version is available at https://doi.org/10.1053/j.ajkd.2024.01.363.
Copyright © 2024.
Abstract
IgA nephropathy is the most prevalent primary glomerulonephritis. Risk factors include tobacco use, male sex, and Asian or European descent. Common presentations include hematuria, non-nephrotic range proteinuria, and slow disease progression. Tx is typically conservative, but immunosuppressive therapy may be considered for those at high risk for rapidly progressive disease. We report a case of a 36 yo male with PMH of uncontrolled HTN presenting to the ED for acute chest pain and SOB. He was admitted for PE's diagnosed on CT, and nephrology was consulted for elevated Cr and renal US consistent with some CKD. The pt had no known baseline Cr due to lack of medical follow-up, and he had no known history of kidney disease. UA was positive for gross hematuria and non-nephrotic range proteinuria. Microscopy showed diffuse non dysmorphic RBCs but no RBC casts on initial evaluation. Renal serologies were negative, and the pt had no hx of NSAID use, illicit drug use, or recent infectious illness. Renal biopsy described diffuse proliferative and sclerosing glomerulonephritis consistent with IgA nephropathy. Per KDIGO guidelines, immunosuppression for IgA nephropathy is considered on a pt specific basis, and those with RPGN should be offered tx in accordance with AAV guidelines. Biopsy results reported MEST-C score of Ml, E1, S1, T1, C2 with moderate chronicity. Per IgA international prediction tool score, the pt had a 67% risk of 50% decline in eGFR or progression to ESRD in 5 years. He was started on IV solumedrol lg x 3 days and d/c on oral prednisone lmg/kg. Infectious etiology was ruled out with blood cultures and echo prior to immunosuppression. Tx also included PCP prophylaxis, Vit D, and Ca supplements. Cr was downtrending upon discharge, and he was scheduled for outpt nephrology f/u. While most cases of IgA nephropathy are slow progressing, we report a patient at significant risk for rapid disease progression. In addition, the disease coincided with unexplained, acute pulmonary emboli and serves as a rare presentation of IgA nephropathy.