| Literature DB >> 26984885 |
Nils Landegren1, Mina Pourmousa Lindberg2, Jakob Skov3, Åsa Hallgren3, Daniel Eriksson3, Trine Lisberg Toft-Bertelsen4, Nanna MacAulay4, Eva Hagforsen2, Anne Räisänen-Sokolowski5, Heikki Saha6, Thomas Nilsson2, Gunnel Nordmark2, Sophie Ohlsson7, Jan Gustafsson8, Eystein S Husebye9, Erik Larsson10, Mark S Anderson11, Jaakko Perheentupa12, Fredrik Rorsman2, Robert A Fenton13, Olle Kämpe3.
Abstract
Tubulointerstitial nephritis is a common cause of kidney failure and may have diverse etiologies. This form of nephritis is sometimes associated with autoimmune disease, but the role of autoimmune mechanisms in disease development is not well understood. Here, we present the cases of three patients with autoimmune polyendocrine syndrome type 1 who developed tubulointerstitial nephritis and ESRD in association with autoantibodies against kidney collecting duct cells. One of the patients developed autoantibodies targeting the collecting duct-specific water channel aquaporin 2, whereas autoantibodies of the two other patients reacted against the HOXB7 or NFAT5 transcription factors, which regulate the aquaporin 2 promoter. Our findings suggest that tubulointerstitial nephritis developed in these patients as a result of an autoimmune insult on the kidney collecting duct cells.Entities:
Keywords: Immunology and pathology; end stage kidney disease; interstitial fibrosis
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Year: 2016 PMID： 26984885 PMCID： PMC5042672 DOI： 10.1681/ASN.2015101126
Source DB: PubMed Journal: J Am Soc Nephrol ISSN： 1046-6673 Impact factor: 10.121