Outcomes of Initial Management Strategies in Patients With Autoimmune Lymphocytic Hypophysitis: A Systematic Review and Meta-analysis

Donegan D., Saeed Z., Delivanis D. A. , Murad M. H. , Honegger J., Amereller F., ...More

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, vol.107, no.4, pp.1170-1190, 2022 (Peer-Reviewed Journal) identifier identifier identifier

  • Publication Type: Article / Review
  • Volume: 107 Issue: 4
  • Publication Date: 2022
  • Doi Number: 10.1210/clinem/dgab839
  • Journal Indexes: Science Citation Index Expanded, Scopus, PASCAL, Agricultural & Environmental Science Database, BIOSIS, CAB Abstracts, Chemical Abstracts Core, CINAHL, EMBASE, Food Science & Technology Abstracts, Gender Studies Database, MEDLINE, Veterinary Science Database
  • Page Numbers: pp.1170-1190
  • Keywords: adenohypophysitis, infundibuloneurohypophysitis, panhypophysitis, autoimmunity, pituitary, treatment, SINGLE-CENTER, CLINICAL CHARACTERISTICS, DIABETES-INSIPIDUS, CASE SERIES, DIAGNOSIS, EXPERIENCE


Context Lymphocytic hypophysitis (LyHy) is characterized by inflammation of the pituitary and or neuroinfundibulum and is uncommon. Treatment options include observation, high-dose glucocorticoids (HD-GCs) or surgery. Optimal first-line management strategy, however, remains unknown. Objective This work aimed to assess response to first-line treatment options (observation, HD-GCs, or surgery) of clinically relevant outcomes (symptomatic, hormonal, and radiographic improvement) among patients with LyHy. Methods A systematic review was conducted in 6 databases through 2020. Meta-analysis was conducted when feasible using a random-effects model. Results We included 33 studies reporting on 591 patients (423 women, 72%) with LyHy. Improvement/resolution of anterior pituitary dysfunction was highest when HD-GCs was first-line treatment. Surgery was associated with the greatest proportion of patients who had regression on imaging. Subgroup analysis comparing HD-GCs to observation showed the odds of anterior pituitary hormone recovery (OR 3.41; 95% CI, 1.68-6.94) or radiographic regression (OR 3.13; 95% CI, 1.54-6.36) were higher with HD-GCs, but so was the need for additional forms of treatment (OR 4.37; 95% CI, 1.70-11.22). No statistically significant difference was seen in recovery of diabetes insipidus (OR 0.9; 95% CI, 0.26-3.10). Certainty in these estimates was very low. Conclusion Observation and use of HD-GCs both are successful first-line management strategies in LyHy. Although use of HD-GCs was associated with increased recovery of anterior pituitary hormone deficit, it also was associated with greater likelihood of additional treatment after withdrawal. Optimal dosing and duration of HD-GCs remains unknown.