Hypophysitis: a single-center case series.

Abstract

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.