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Lymphocytic hypophysitis with diabetes insipidus in a young man

Nature Reviews Endocrinology volume 6, pages 464–470 (2010)Cite this article

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Abstract

Background. A 29-year-old man was referred to a multidisciplinary pituitary clinic with a 3.5-year history of central diabetes insipidus, initially presumed to be idiopathic based on a normal MRI scan of the pituitary gland. Subsequent scanning revealed a suprasellar mass, which demonstrated progressive enlargement on serial imaging. He also developed hypogonadotropic hypogonadism.

Investigations. Measurement of levels of serum morning fasting cortisol, adrenocorticotropic hormone, total testosterone, luteinizing hormone, follicle-stimulating hormone, prolactin, insulin-like growth factor 1, TSH and free T4, MRI of the pituitary gland and a transsphenoidal biopsy of a pituitary mass were performed.

Diagnosis. Lymphocytic hypophysitis presenting with diabetes insipidus, with development of hypogonadotropic hypogonadism and a suprasellar mass.

Management. The patient was treated with intranasal desmopressin and transdermal testosterone. The underlying lymphocytic hypophysitis was initially managed conservatively with serial MRI and visual field testing. No immunosuppressant medication was given and, aside from the diagnostic transsphenoidal biopsy, no surgical intervention was required. He subsequently developed secondary hypothyroidism, secondary adrenal insufficiency and growth hormone deficiency. These disorders were managed with levothyroxine and prednisone.

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Figure 1: MRI scan of the pituitary of the case patient.
Figure 2: Histopathology images from a biopsy sample of the case patient's pituitary gland.

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Acknowledgements

Written consent for publication was obtained from the patient. Charles P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the MedscapeCME-accredited continuing medical education activity associated with this article.

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Authors and Affiliations

  1. Department of Medicine, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, 02115, MA, USA

    Ole-Petter R. Hamnvik & Ursula B. Kaiser

  2. Department of Pathology, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, 02115, MA, USA

    Anna R. Laury

  3. Department of Neurosurgery, Brigham and Women's Hospital and Harvard Medical School, 75 Francis Street, Boston, 02115, MA, USA

    Edward R. Laws Jr

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  1. Ole-Petter R. Hamnvik
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Correspondence to Ursula B. Kaiser.

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Hamnvik, OP., Laury, A., Laws Jr, E. et al. Lymphocytic hypophysitis with diabetes insipidus in a young man. Nat Rev Endocrinol 6, 464–470 (2010). https://doi.org/10.1038/nrendo.2010.104

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