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dc.contributor.authorYuen, Kevin C. J.
dc.contributor.authorMoloney, Kelley J.
dc.contributor.authorMercado, Jennifer U.
dc.contributor.authorRostad, Steven
dc.contributor.authorMcCullough, Brendan J.
dc.contributor.authorLitvack, Zachary N.
dc.contributor.authorDelashaw, Johnny B.
dc.contributor.authorMayberg, Marc R.
dc.date.accessioned2018-07-24T23:54:06Z
dc.date.available2018-07-24T23:54:06Z
dc.date.issued2018-06
dc.identifier.citationYuen, K.C.J., Moloney, K.J., Mercado, J.U. et al. Pituitary (2018) 21: 238. https://doi.org/10.1007/s11102-017-0852-4en_US
dc.identifier.issn1386-341X
dc.identifier.issn1573-7403
dc.identifier.pmid29248982
dc.identifier.doi10.1007/s11102-017-0852-4
dc.identifier.urihttp://hdl.handle.net/10150/628298
dc.description.abstractBackground IgG4-related hypophysitis is a rare clinical entity that forms part of an emerging group of multi-organ IgG4-related fibrosclerotic systemic diseases. The rare prevalence of the disease, presenting features that overlap with other sellar pathologies, and variable imaging features can make preoperative identification challenging. Purpose and methods We report three cases of isolated IgG4-related hypophysitis with atypical clinical and imaging features that mimicked those of pituitary apoplexy and other sellar lesions. Additionally, we review the literature of IgG4-related hypophysitis to provide context for individual patient data described herein. Results All patients presented with symptoms that mimicked those of pituitary apoplexy and visual disturbance, and MRI findings suggestive of pituitary macroadenoma, Rathke's cleft cyst and craniopharyngioma. The clinical presentation warranted surgical decompression, resulting in rapid symptomatic improvement. Preoperative high-dose followed by postoperative low-dose glucocorticoid replacement therapy was administered in all cases. Histopathology showed dense infiltrate of IgG4 cells. Post-operative follow-up monitoring for 12-26 months revealed normal serum IgG4 levels with no other organ involvement, while endocrinological testing revealed persistent pituitary hormone deficiencies. Conclusions Our cases highlight the importance of considering IgG4-related hypophysitis in the differential diagnosis of solid and cystic sellar lesions presenting acutely with pituitary apoplexy symptoms. Existing diagnostic criteria may not be sufficiently precise to permit rapid and reliable identification, or avoidance of surgery in the acute setting. In contrast to other reports of the natural history of this condition, despite the severity of presenting features, the disease in our cases was pituitary-restricted with normal serum IgG4 levels.en_US
dc.language.isoenen_US
dc.publisherSPRINGERen_US
dc.relation.urlhttp://link.springer.com/10.1007/s11102-017-0852-4en_US
dc.rights© Springer Science+Business Media, LLC, part of Springer Nature 2017en_US
dc.subjectIgG4-related hypophysitisen_US
dc.subjectAtypicalen_US
dc.subjectImagingen_US
dc.subjectIgG4en_US
dc.subjectRathke's cleft cysten_US
dc.subjectCraniopharyngiomaen_US
dc.titleA case series of atypical features of patients with biopsy-proven isolated IgG4-related hypophysitis and normal serum IgG4 levelsen_US
dc.typeArticleen_US
dc.contributor.departmentUniv Arizona, Coll Med, Dept Neuroendocrinolen_US
dc.contributor.departmentUniv Arizona, Coll Med, Dept Neurosurgen_US
dc.identifier.journalPITUITARYen_US
dc.description.note12 month embargo; published online: 16 December 2017en_US
dc.description.collectioninformationThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at repository@u.library.arizona.edu.en_US
dc.eprint.versionFinal accepted manuscripten_US
dc.source.journaltitlePituitary
dc.source.volume21
dc.source.issue3
dc.source.beginpage238
dc.source.endpage246


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