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Pituitary hypophysitis in granulomatosis with polyangiitis (GPA): a case series

  • Majid Alameri,
  • Abdulla Alnuaimi,
  • Niamh M. Martin,
  • Karim Meeran,
  • Anastasia Gontsarova,
  • Tara D. Barwick,
  • Spencer Ellis,
  • Stephen McAdoo,
  • James Tomlinson,
  • Florian Wernig

摘要

Granulomatosis with polyangiitis (GPA) rarely involves the pituitary gland. Pituitary involvement has been reported in ~ 1% of all cases of GPA. Most commonly, pituitary swelling and inflammation results in symptoms due to pituitary mass effect and arginine vasopressin deficiency. To date, there are no pituitary-specific treatment guidelines for this rare condition. We present three patients with GPA-related hypophysitis highlighting the spectrum of pituitary involvement. All three patients were successfully treated with immunosuppressive regimens that included rituximab (RTX). Following remission induction with high-dose glucocorticoids, patients received 6 monthly RTX for remission maintenance. RTX was well tolerated without significant side effects.