{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T14:17:26Z","timestamp":1784557046107,"version":"3.55.0"},"reference-count":9,"publisher":"Bioscientifica","issue":"2","license":[{"start":{"date-parts":[[2023,4,1]],"date-time":"2023-04-01T00:00:00Z","timestamp":1680307200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["edm.bioscientifica.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,4,1]]},"abstract":"\n Summary<\/jats:title>\n We report a rare case of biopsy-proven isolated immunoglobulin G4 (IgG4)-related hypophysitis and Rathke\u2019s cleft cyst (RCC) presenting as panhypopituitarism. A 54-year-old Caucasian female presented with symptoms of slurred speech, altered mental status, polyuria and polydipsia and was found to have panhypopituitarism. Brain MRI showed a suprasellar mass with suspected intralesional hemorrhage. She underwent trans-sphenoidal resection due to MRI evidence of compression of the optic chiasm and left optic nerve. Preoperatively, she was started on hydrocortisone, levothyroxine and desmopressin. Histopathology demonstrated a RCC with adjacent lymphoplasmacytic hypophysitis with numerous IgG4-immunoreactive plasma cells. Hydrocortisone was stopped at 10 months after confirming hypothalamic-pituitary-adrenal (HPA)-axis recovery and desmopressin was stopped at 1 year. There was recurrence of a cystic mass at 1 year follow-up. Over 4 years of follow-up, she continued to require levothyroxine, and the mass remained stable in size. In order to begin to understand how this case\u2019s unique histopathological presentation influences clinical presentation, pituitary imaging and prognosis, we present an accompanying literature review.<\/jats:p>\n <\/jats:sec>\n \n Learning points<\/jats:title>\n \n \n Isolated IgG4 hypophysitis and coexisting Rathke\u2019s cleft cyst is a rare condition, which presents a diagnostic challenge.<\/jats:p>\n <\/jats:list-item>\n \n Recognizing its characteristic features can assist with early recognition and initiation of therapy to promote optimal outcomes.<\/jats:p>\n <\/jats:list-item>\n \n Further studies investigating the mechanisms promoting co-occurrence of these entities and their effect on prognosis are needed.<\/jats:p>\n <\/jats:list-item>\n <\/jats:list>\n <\/jats:sec>","DOI":"10.1530\/edm-22-0359","type":"journal-article","created":{"date-parts":[[2023,4,17]],"date-time":"2023-04-17T08:37:52Z","timestamp":1681720672000},"update-policy":"https:\/\/doi.org\/10.1530\/crossmarkpolicy-2","source":"Crossref","is-referenced-by-count":0,"title":["A rare case of isolated IgG4-related hypophysitis with Rathke\u2019s cleft cyst presenting as panhypopituitarism"],"prefix":"10.1530","volume":"2023","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2598-2402","authenticated-orcid":true,"given":"Hiba Z","family":"Hashmi","sequence":"first","affiliation":[{"name":"Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dinkar","family":"Rupakula","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Diabetes and Metabolism, University of Arizona, Tucson, Arizona, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rekha","family":"Magar","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"H Brent","family":"Clark","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine and Pathology, University of Minnesota, Minneapolis, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amir","family":"Moheet","sequence":"additional","affiliation":[{"name":"Division of Endocrinology, Diabetes and Metabolism, University of Minnesota, Minneapolis, Minnesota, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"416","reference":[{"key":"bib1","series-title":"Brain Pathology","first-page":"839","article-title":"IgG4-related hypophysitis is highly prevalent among cases of histologically confirmed hypophysitis","volume":"27","author":"Bernreuther","year":"2017","unstructured":"Bernreuther CIllies CFlitsch JBuchfelder MBuslei RGlatzel M & Saeger W. IgG4-related hypophysitis is highly prevalent among cases of histologically confirmed hypophysitis. Brain Pathology201727839\u2013845. (https:\/\/doi.org\/10.1111\/BPA.12459)"},{"key":"bib2","series-title":"World Neurosurgery","first-page":"735","article-title":"Clinical interrogation for unveiling an isolated hypophysitis mimicking pituitary adenoma","volume":"99","author":"Lee","year":"2017","unstructured":"Lee SChoi JHKim CJ & Kim JH. Clinical interrogation for unveiling an isolated hypophysitis mimicking pituitary adenoma. World Neurosurgery201799735\u2013744. (https:\/\/doi.org\/10.1016\/J.WNEU.2016.07.071)"},{"key":"bib3","series-title":"Journal of Clinical Endocrinology and Metabolism","article-title":"IgG4-related hypophysitis: a new addition to the hypophysitis spectrum","volume":"96","author":"Leporati","year":"2011","unstructured":"Leporati PLandek-Salgado MALupi IChiovato L & Caturegli P. IgG4-related hypophysitis: a new addition to the hypophysitis spectrum. Journal of Clinical Endocrinology and Metabolism201196 1971\u20131980. (https:\/\/doi.org\/10.1210\/JC.2010-2970)"},{"key":"bib4","series-title":"Asian Journal of Surgery","first-page":"93","article-title":"IgG4-related hypophysitis presenting as a pituitary adenoma with systemic disease","volume":"36","author":"Hsing","year":"2013","unstructured":"Hsing MTHsu HTCheng CY & Chen CM. IgG4-related hypophysitis presenting as a pituitary adenoma with systemic disease. Asian Journal of Surgery20133693\u201397. (https:\/\/doi.org\/10.1016\/J.ASJSUR.2012.04.013)"},{"key":"bib5","series-title":"Pituitary","first-page":"282","article-title":"Critical review of IgG4-related hypophysitis","volume":"20","author":"Shikuma","year":"2017","unstructured":"Shikuma JKan KIto RHara KSakai HMiwa TKanazawa A & Odawara M. Critical review of IgG4-related hypophysitis. Pituitary201720282\u2013291. (https:\/\/doi.org\/10.1007\/s11102-016-0773-7)"},{"key":"bib6","series-title":"Pituitary","first-page":"238","article-title":"A case series of atypical features of patients with biopsy-proven isolated IgG4-related hypophysitis and normal serum IgG4 levels","volume":"21","author":"Yuen","year":"2018","unstructured":"Yuen KCJMoloney KJMercado JURostad SMcCullough BJLitvack ZNDelashaw JB & Mayberg MR. A case series of atypical features of patients with biopsy-proven isolated IgG4-related hypophysitis and normal serum IgG4 levels. Pituitary201821238\u2013246. (https:\/\/doi.org\/10.1007\/s11102-017-0852-4)"},{"key":"bib7","series-title":"Neurosurg","first-page":"332","article-title":"Turk Pure lymphocytic \u0131nfundibuloneurohypophysitis caused by the rupture of Rathke\u2019s cleft cyst: report of 2 cases and review of the literature Rathke Yar\u0131\u011f\u0131 Kisti R\u00fcpt\u00fcr\u00fc nedeniyle saf Lenfositik \u0130nfundibulon\u00f6rohipofizit: \u0130ki olgu sunumu ve Literat\u00fcr Derlemesi","volume":"25","author":"Hayash\u0131","year":"2015","unstructured":"Hayash\u0131 YO\u0131sh\u0131 MK\u0131ta DWatanabe TTach\u0131bana O & Hamada JI. Turk Pure lymphocytic \u0131nfundibuloneurohypophysitis caused by the rupture of Rathke\u2019s cleft cyst: report of 2 cases and review of the literature Rathke Yar\u0131\u011f\u0131 Kisti R\u00fcpt\u00fcr\u00fc nedeniyle saf Lenfositik \u0130nfundibulon\u00f6rohipofizit: \u0130ki olgu sunumu ve Literat\u00fcr Derlemesi. Neurosurg201525332\u2013336. (https:\/\/doi.org\/10.5137\/1019-5149.JTN.9776-13.0)"},{"key":"bib8","series-title":"Journal of Neuroendocrinology","article-title":"Histopathological findings in the landscape of IgG4-related pathology in patients with pituitary dysfunction: review of six cases","volume":"33","author":"Vasaitis","year":"2021","unstructured":"Vasaitis LWikstr\u00f6m JAhlstr\u00f6m SGudjonsson OKumlien EEd\u00e9n Engstr\u00f6m B & Casar-Borota O. Histopathological findings in the landscape of IgG4-related pathology in patients with pituitary dysfunction: review of six cases. Journal of Neuroendocrinology202133 e12942. (https:\/\/doi.org\/10.1111\/JNE.12942)"},{"key":"bib9","series-title":"Neurosurgical Review","first-page":"157","article-title":"Rathke\u2019s cleft cyst rupture as potential initial event of a secondary perifocal lymphocytic hypophysitis: proposal of an unusual pathogenetic event and review of the literature","volume":"31","author":"Schittenhelm","year":"2008","unstructured":"Schittenhelm JBeschorner RPsaras TCapper DN\u00e4gele TMeyermann RSaeger WHonegger J & Mittelbronn M. Rathke\u2019s cleft cyst rupture as potential initial event of a secondary perifocal lymphocytic hypophysitis: proposal of an unusual pathogenetic event and review of the literature. Neurosurgical Review200831157\u2013163. 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