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

Research output: Contribution to journalJournal articleResearchpeer-review

  • Nils Landegren
  • Mina Pourmousa Lindberg
  • Jakob Skov
  • Åsa Hallgren
  • Daniel Eriksson
  • Eva Hagforsen
  • Anne Räisänen-Sokolowski
  • Heikki Saha
  • Thomas Nilsson
  • Gunnel Nordmark
  • Sophie Ohlsson
  • Jan Gustafsson
  • Eystein S Husebye
  • Erik Larsson
  • Mark S Anderson
  • Jaakko Perheentupa
  • Fredrik Rorsman
  • Robert A Fenton
  • Olle Kämpe
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.
Original languageEnglish
JournalJournal of the American Society of Nephrology:JASN
Issue number10
Pages (from-to)3220-3228
Publication statusPublished - Oct 2016

ID: 161739491