Hypophysitis in the era of immune checkpoint inhibitors and immunoglobulin G4-related disease

Leen Wehbeh, Sama Alreddawi, Roberto Salvatori

Research output: Contribution to journalReview article

Abstract

Introduction: Hypophysitis is a rare disorder, defined as inflammation of the pituitary gland that may result in pituitary enlargement and varying anterior and posterior pituitary hormonal deficits. It involves different histopathological subtypes and variable etiologies, with considerable overlap between classification systems. Histopathology is the gold standard diagnostic approach. Areas covered: In this article, we will review the major histopathological subtypes of hypophysitis with a special focus on immunoglobulin G4 (IgG4)-related hypophysitis and immune checkpoint inhibitor-induced hypophysitis, given their recent appearance and increasing incidence. We will summarize the similarities and differences between the different subtypes as it relates to epidemiology, pathogenesis, presentation, diagnosis, and management. Expert opinion: Hypophysitis is a heterogeneous and wide term used to describe different, possibly distinct diseases often with poorly understood pathogenesis. It involves a wide range of subtypes with certain differences in incidence rates, pathogenesis, and management. Management usually focuses on relieving the mass effect symptoms and replacing the deficient pituitary hormones. Spontaneous recovery is possible but recurrence is not uncommon.

Original languageEnglish (US)
Pages (from-to)167-178
Number of pages12
JournalExpert Review of Endocrinology and Metabolism
Volume14
Issue number3
DOIs
StatePublished - May 4 2019

Keywords

  • Autoimmune
  • CTLA-4
  • IgG4
  • granulomatous
  • hypophysitis
  • immune checkpoint inhibitors
  • immunoglobulin G4
  • lymphocytic
  • xanthomatous

ASJC Scopus subject areas

  • Endocrinology, Diabetes and Metabolism

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