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

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.

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.

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.