Hypophysitis: clinical experience in an Australian case series

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 130-162-Neuroendocrinology
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-140
Monique Louise Costin*1, Vicki Maltby2, Anna D Duke3, Mark McLean4, Amy Wagstaff5, David Chipps6, Andrew John Weissberger7, Roderick John Clifton-Bligh8, Suja Padmanabhan6, Aidan McElduff9, Jane Holmes-Walker10, Weiwen Chen11, Rachel Anne Bradbury12, Richard Harvey13, Patricia Anne Crock14, Ganesh Chockalingam15, Katherine Samaras16, Peter Earls1 and Ann I McCormack17
1St Vincent's Hospital, Australia, 2John Hunter Hospital, Australia, 3Westmead Hospital, Beecroft NSW, Australia, 4Univ of Western Sydney, Sydney NSW, Australia, 5Westmead Hospital, Sydney, Australia, 6Westmead Hospital, Australia, 7St Vincent's Clinic, Darlinghurst NSW, Australia, 8Royal North Shore Hospital, Sydney, Australia, 9Northern Sydney Endocrine Centre, NSW, Australia, 10Westmead Hosp, North Sydney, NS, Australia, 11Garvan Institute of Medical Research and St Vincent's Hospital, Sydney, Australia, 12Concord Hospital, Sydney NSW, Australia, 13Sydney Ear Nose and Throat Clinic, NSW, Australia, 14John Hunter Children's Hosp, Newcastle NSW, Australia, 15Canberra Hospital, ACT, Australia, 16St Vincent's Hospital, Sydney NSW, Australia, 17St. Vincent Hospital, Naremburn - NSW, Australia
Introduction: Hypophysitis, an inflammatory condition of the pituitary, is uncommon and can be difficult to diagnose clinically. Knowledge remains limited regarding its natural history and the best approach to management. Methods: A multi-center clinical case series was assembled. Hypophysitis was diagnosed histopathologically or clinically in the presence of: hypopituitarism, DI, sella/pituitary stalk-based mass on MRI, autoimmune history, positive pituitary antibodies or ipilimumab use. Results: Data was collected on 20 patients (6 men, 14 women): 14 had histologically-confirmed hypophysitis, 6 clinically-diagnosed. Average follow-up was 61 months. Hypopituitarism-related symptoms was the most common presentation, confirmed in 19 patients: 11 had 2-3 affected axes, 4 isolated ACTH deficiency and 6 DI. Headache was common (12), with abnormal vision in 5. 9 patients had an autoimmune history. Pituitary antibodies were tested in 2 patients, both positive, one with no known autoimmune disease. A mass lesion, commonly enhancing, was the most frequent radiologic abnormality (14), with an enlarged pituitary in 5. 7 patients had stalk thickening with a dural tail in 3. One patient with ipilimumab use had no significant MRI abnormalities. 14 patients underwent pituitary surgery. Hypophysitis was suspected preoperatively in only 4; the remaining patients had a presumed macroadenoma. Histopathological-diagnoses were lymphocytic (10), granulomatous (3) and xanthogranulomatous (1). Postoperatively, headaches improved in 9/10, pituitary function improved in only 2, radiologic changes improved in 5/10. 3 patients had symptomatic relapses treated with supra-physiological corticosteroids; in 2 with multiple relapses steroid-sparing agents were successfully used. 10 of 11 with long-term control received only replacement corticosteroid doses. The 6 patients with clinically-diagnosed hypophysitis had less severe clinical presentation and more subtle MRI findings.  Apart from 1 case with DI alone, all had ACTH deficiency with other affected axes in 3. Symptoms resolved in all patients with only pituitary replacement therapy. 2 patients with progress imaging had no progression. Conclusions: In clinically-suspected hypophysitis, patients appear to do well with pituitary replacement therapy alone. Patients with mass lesions may benefit from surgical debulking for symptom relief with supra-physiological and steroid-sparing agents reserved for the minority with relapsing disease.

Nothing to Disclose: MLC, VM, ADD, MM, AW, DC, AJW, RJC, SP, AM, JH, WC, RAB, RH, PAC, GC, KS, PE, AIM

*Please take note of The Endocrine Society's News Embargo Policy at http://www.endo-society.org/endo2013/media.cfm