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A Potpourri of Cases from the Collection of Dr. Bijol

Updated: Jun 7, 2023


GLOMERULAR DISEASE DIAGNOSIS



In this session of Nephropathology Essentials, Dr. Bijol utilized a case-based approach to review a variety of important nephropathology topics. Our Moderator’s Notes are derived from her live presentation.





By Dr. Pravir Baxi




Key points:

  • Rarely, patients can have chronic, indolent glomerulonephritis that may be undiagnosed due to the absence of overt nephrotic/nephritic findings (e.g., asymptomatic, non-nephrotic proteinuria). These patients can potentially progress to have clinically significant renal disease.

  • Histologically active lesions on a renal biopsy can be missed or appear absent due to the focal nature of the disease and/or a result of a sampling error (e.g., medulla versus cortex). This can lead to delay in diagnosis or inadequate treatment, leading to an increased risk of CKD/ESKD.

  • Drug-induced vasculitis

    • In patients with pauci-immune (+ANCA) crescentic glomerulonephritis (GN), a complete search for secondary causes, including medications and infections, is recommended.

    • Common medications: hydralazine, allopurinol, anti-thyroid drugs (propylthiouracil>>methimazole), minocycline, penicillamine, levamisole

    • Typically, these patients will have very high MPO or PR3 levels, elevated ANA, low C3/C4, or atypical features on histopathology, such as low-level Ig deposition.

  • Vascular endothelial grow factor (VEGF) inhibition

    • VEGF inhibitors act through various mechanisms (via numerous downstream signaling pathways) and can lead to nephrotoxicity.

    • Most common renal presentations related to these agents are hypertension, proteinuria (which can progress to full nephrotic syndrome), and thrombotic microangiopathy (TMA). ATN has also been described.

    • On renal biopsy, common histologic findings include endotheliosis, podocytopathy, TMA, and MPGN-pattern of injury.





Selected References:

  1. Estrada CC, Maldonado A, Mallipattu S. Therapeutic Inhibition of VEGF Signaling and Associated Nephrotoxicities. J Am Soc Nephrol. 2019. https://www.ncbi.nlm.nih.gov/pubmed/?term=30642877

  2. Hacking S, Uppal NN, Khan N, Ionescu M, and Bijol V. Systemic p-ANCA vasculitis with fatal outcome, arising in the setting of methimazole use. Clin Nephrol Case Stud. 2019. https://www.ncbi.nlm.nih.gov/pubmed/31008018

  3. Radhakrishnan J, Perazella MA. Drug-induced glomerular disease: attention required! Clin J Am Soc Nephrol. 2015. https://www.ncbi.nlm.nih.gov/pubmed/25876771



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