The authors review their treatment experience and summarize clinical outcomes for patients with hypophysitis over the past 15 years.
A retrospective analysis was conducted on patients with lymphocytic, granulomatous or IgG4-related hypophysitis treated from 1997 to 2014 at a single academic center. Patients’ medical records were reviewed and binary logistic regression analysis was used to assess whether various clinical parameters were associated with improved outcomes including endocrine function, radiographic appearance and disease recurrence.
Twenty-one patients (13 women and 8 men) were identified with a median diagnosis age of 37.4 years. All but two patients (90 %) were diagnosed histopathologically and the remaining two were diagnosed clinically with lymphocytic hypophysitis. 16 patients (76 %) had lymphocytic hypophysitis, 3 (14 %) had granulomatous hypophysitis, 1 (5 %) had IgG4-related hypophysitis and 1 (5 %) had mixed lymphocytic-granulomatous. Patients presented with various symptoms of expanding sellar mass with most common signs including headache (57 %), polyuria/polydipsia (52 %), vision changes (52 %) and amenorrhea or decreased libido (48 %). Pre-treatment endocrine evaluation revealed that 12 (57 %) patients had complete anterior hypopituitarism, 11 patients (52 %) had diabetes insipidus, ten patients (48 %) had mild hyperprolactinemia and three patients (14 %) had isolated endocrine axis deficiencies with partial gland function. We observed a broad diversity in pre-treatment imaging with common findings including uniform contrast enhancement (62 %), thickened infundibulum (57 %) and loss of hypophysis bright spot on T1 imaging (43 %). Patients were treated with steroids and hormone supplementation as needed. 16 patients (76 %) had recorded post-treatment MRI scans which revealed that half had radiographic improvement and half had stable or worsened post-treatment imaging. Only female gender was found to significantly predict improved odds of post-steroid radiographic improvement. For post-treatment endocrine evaluation, six patients (29 %) did not have an evaluation on record, four patients (19 %) had some improvement in at least one axis, seven patients (33 %) had stable but non-worsened endocrine function and four patients (19 %) had worsened endocrine function post-steroids.
Hypophysitis is an increasingly recognized diagnosis that can present with a broad array of radiographic and clinical features. Surgical biopsy can be helpful to make definitive diagnosis and may guide treatment decision-making.
KeywordsAutoimmune hypophysitis Pituitary inflammation Sellar mass Stalk thickening Granulomatous Lymphocytic
We thank Jeffrey Wagner, BS for his assistance with identification of cases.
Conflict of interest
The authors declare that they have no conflict of interest.
- 5.Khare S, Jagtap VS, Budyal SR et al (2013) Primary (autoimmune) hypophysitis: a single centre experience. PituitaryGoogle Scholar
- 12.De Bellis A, Dello Iacovo A, Bellastella G et al (2014) Characterization of pituitary cells targeted by antipituitary antibodies in patients with isolated autoimmune diseases without pituitary insufficiency may help to foresee the kind of future hypopituitarism. Pituitary 17:457–463Google Scholar
- 18.Honegger J, Fahlbusch R, Bornemann A et al (1997) Lymphocytic and granulomatous hypophysitis: experience with nine cases. Neurosurgery 40:713–722. Discussion 722–723Google Scholar
- 21.Menon SK, Sarathi V, Bandgar TR et al (2009) Autoimmune hypophysitis: a single centre experience. Singap Med J 50:1080–1084Google Scholar
- 29.Buxton N, Robertson I (2001) Lymphocytic and granulocytic hypophysitis: a single centre experience. Br J Neurosurg 15:242–245. Discussion 245–246Google Scholar
- 58.Howlett TA, Levy MJ, Robertson IJ (2010) How reliably can autoimmune hypophysitis be diagnosed without pituitary biopsy. Clin Endocrinol (Oxf) 73:18–21Google Scholar
- 60.Jahangiri A, Wagner JR, Pekmezci M et al (2013) A comprehensive long-term retrospective analysis of silent corticotrophic adenomas vs hormone-negative adenomas. Neurosurgery 73:8–17. Discussion 17–18Google Scholar
- 61.Jahangiri A, Wagner J, Han SW et al (2014) Morbidity of repeat transsphenoidal surgery assessed in more than 1000 operations. J Neurosurg 121:67–74Google Scholar