Endocrinol Metab.  2014 Dec;29(4):470-478. 10.3803/EnM.2014.29.4.470.

Clinical Characteristics, Management, and Outcome of 22 Cases of Primary Hypophysitis

• Park SM
• Bae JC
• Joung JY
• Cho YY
• Kim TH
• Jin SM
• Suh S
• Hur KY
• Kim KW

• KMID: 2384244
• DOI: http://doi.org/10.3803/EnM.2014.29.4.470

Abstract

BACKGROUND
Primary hypophysitis causes varying degrees of endocrine dysfunction and mass effect. The natural course and best treatment have not been well established.
METHODS
Medical records of 22 patients who had been diagnosed with primary hypophysitis between January 2001 and March 2013 were retrospectively reviewed. Based on the anatomical location, we classified the cases as adenohypophysitis (AH), infundibuloneurohypophysitis (INH), and panhypophysitis (PH). Clinical presentation, endocrine function, pathologic findings, magnetic resonance imaging findings, and treatment courses were reviewed.
RESULTS
Among 22 patients with primary hypophysitis, 81.8% (18/22) had involvement of the posterior pituitary lobe. Two patients of the AH (2/3, 66.6%) and three patients of the PH (3/10, 30%) groups initially underwent surgical mass reduction. Five patients, including three of the PH (3/10, 33.3%) group and one from each of the AH (1/3, 33.3%) and INH (1/9, 11.1%) groups, initially received high-dose glucocorticoid treatment. Nearly all of the patients treated with surgery or high-dose steroid treatment (9/11, 82%) required continuous hormone replacement during the follow-up period. Twelve patients received no treatment for mass reduction due to the absence of acute symptoms and signs related to a compressive mass effect. Most of them (11/12, 92%) did not show disease progression, and three patients recovered partially from hormone deficiency.
CONCLUSION
Deficits of the posterior pituitary were the most common features in our cases of primary hypophysitis. Pituitary endocrine defects responded less favorably to glucocorticoid treatment and surgery. In the absence of symptoms related to mass effect and with the mild defect of endocrine function, it may not require treatment to reduce mass except hormone replacement.

Keyword

Pituitary; Hypophysitis; Diabetes insipidus; Hypopituitarism; Steroids

MeSH Terms

Diabetes Insipidus
Disease Progression
Follow-Up Studies
Humans
Hydrogen-Ion Concentration
Hypopituitarism
Magnetic Resonance Imaging
Medical Records
Retrospective Studies
Steroids
Steroids

Figure

• 

  Fig. 1 Summary of treatment courses of 22 patients with primary hypophysitis. TSA, transsphenoidal approach. aDue to regrowth of mass; bDue to steroid related side effect and progress with steroid tapering; cDue to progression in mass size.

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