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This study reports the presentation, management and outcomes for patients with histologically proven IgG4-related hypophysitis.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A prospectively maintained electronic database was searched over a 14-year period from 1 January 2007 to 31 December 2020 at a single academic centre to identify all patients with a histological diagnosis of IgG4-RH. A retrospective case note review from electronic health records was conducted for each case to extract data on their presentation, management and outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 8 patients (5 male) with a median age of 51\u00a0years were identified. The most common presenting symptoms were headache (4\/8; 50%), fatigue (3\/8; 37.5%) and visual impairment (2\/8; 25%). Three patients were initially treated with high-dose steroids aiming for reduction of the pituitary mass. However, ultimately all patients underwent transsphenoidal surgery. Post-operative changes included radiological reduction in pituitary mass in all patients that had imaging (7\/7; 100%), improvement in vision (1\/2; 50%), residual thick pituitary stalk (5\/7; 71.4%), persistent anterior hypopituitarism (4\/8; 50%) and panhypopopituitarism including diabetes insipidus (3\/8; 37.5%).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>IgG4-RH is an increasingly recognised entity presenting with a variety of symptoms and signs. Clinical presentation is similar to other forms of hypophysitis. It is therefore important to consider IgG4-RH as a differential and to have a low threshold for pituitary biopsy, the diagnostic gold standard. The diagnosis of IgG4-RH will guide decisions for additional workup for IgG4-related disease, multi-disciplinary team involvement and follow-up.<\/jats:p><\/jats:sec>","DOI":"10.1007\/s00701-022-05231-9","type":"journal-article","created":{"date-parts":[[2022,5,9]],"date-time":"2022-05-09T10:02:56Z","timestamp":1652090576000},"page":"2095-2103","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":16,"title":["IgG4-related hypophysitis: a retrospective cohort study"],"prefix":"10.1007","volume":"164","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1745-0345","authenticated-orcid":false,"given":"R.","family":"Bhargava","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Z.","family":"Hussein","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"N. 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