%0 Journal Article %T IgG4-Related Kidney Disease: A Curious Case of Interstitial Nephritis with Hypocomplementemia %A Christopher Cheang Han Leo %A Emmett Tsz Yeung Wong %A Manjari Lahiri %A Ming Teh %J Archive of "Case Reports in Nephrology and Dialysis". %D 2019 %R 10.1159/000500296 %X 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 %K Tubulointerstitial disease %K IgG4-related kidney disease %K Acute kidney injury %K Hypocomplementemia %K Acute interstitial nephritis %U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6547283/