IgG4-related kidney disease has been relatively newly recognized over the last two decades as a combination of an autoimmune and allergic disorder, with elevated serum IgG4 level and hypocomplementemia among its characteristic features. Here we report the case of a man with interstitial nephritis presenting with acute kidney injury and hypocomplementemia but normal serum IgG4 level and provide a literature review of IgG4-related kidney disease. This case highlights the importance of IgG4-related kidney disease as an important differential diagnosis in any patient presenting with a clinical syndrome mimicking acute interstitial nephritis with hypocomplementemia. A high index of suspicion with a low threshold for performing a native kidney biopsy would be paramount as patients do respond well to corticosteroid therapy.
CITATION STYLE
Wong, E. T. Y., Lahiri, M., Teh, M., & Leo, C. C. H. (2019). IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia. Case Reports in Nephrology and Dialysis, 9(2), 49–54. https://doi.org/10.1159/000500296
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