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Autoantibodies Targeting a Collecting Duct-Specific Water Channel in Tubulointerstitial Nephritis

Landegren, Nils; Pourmousa Lindberg, Mina; Skov, Jakob; Hallgren, Åsa; Eriksson, Daniel; Lisberg Toft-Bertelsen, Trine; MacAulay, Nanna; Hagforsen, Eva; Räisänen-Sokolowski, Anne and Saha, Heikki, et al. (2016) In Journal of the American Society of Nephrology : JASN 27(10). p.3220-3228
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... (More)

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.

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@article{ff323006-3b50-4d18-9fa2-7e43d839718e,
  abstract     = {<p>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.</p>},
  author       = {Landegren, Nils and Pourmousa Lindberg, Mina and Skov, Jakob and Hallgren, Åsa and Eriksson, Daniel and Lisberg Toft-Bertelsen, Trine and MacAulay, Nanna and Hagforsen, Eva and Räisänen-Sokolowski, Anne and Saha, Heikki and Nilsson, Thomas and Nordmark, Gunnel and Ohlsson, Sophie and Gustafsson, Jan and Husebye, Eystein S and Larsson, Erik and Anderson, Mark S and Perheentupa, Jaakko and Rorsman, Fredrik and Fenton, Robert A and Kämpe, Olle},
  issn         = {1533-3450},
  keyword      = {Adult,Aquaporins/immunology,Autoantibodies/immunology,Female,Humans,Kidney Tubules, Collecting/immunology,Male,Middle Aged,Nephritis, Interstitial/immunology,Young Adult},
  language     = {eng},
  number       = {10},
  pages        = {3220--3228},
  series       = {Journal of the American Society of Nephrology : JASN},
  title        = {Autoantibodies Targeting a Collecting Duct-Specific Water Channel in Tubulointerstitial Nephritis},
  url          = {http://dx.doi.org/10.1681/ASN.2015101126},
  volume       = {27},
  year         = {2016},
}