Deciphering the Presentation and Etiologies of Hypophysitis Highlights the Need for Repeated Systematical Investigation

Abstract

Abstract Aims Hypophysitis is defined as an inflammation of the pituitary gland and/or infundibulum. Our aim was to characterize the initial course and evolution of patients with hypophysitis according to the different etiologies. Patients and methods Retrospective observational study conducted in a universitary referral hospital center. Patients over 15 years of age were included if they had a diagnosis of hypophysitis between January 2014 and October 2023, with the exclusion of hypophysitis secondary to immune checkpoint inhibitors. Results Sixty-one patients (64% women; median age, 34 years) were included. Polyuria-polydipsia, headache and asthenia were present in 64%, 48% and 44% of cases respectively. At diagnosis, at least one anterior pituitary deficiency was present in 91.5% of cases and vasopressin deficiency in 56%. MRI was abnormal in 97% of cases. Secondary hypophysitis was found in 46% of cases (n=28), including sarcoidosis in 28% (n=17) and L-group histiocytoses in 13.1% (n=8). Among patients with secondary hypophysitis, pituitary deficiency preceded systemic manifestations in 23% and occurred concomitantly in 23% of cases. Patients were treated in 36% of cases (glucocorticoids, surgery…), without improvement of pituitary hormone deficits. Conclusions A systemic etiology of hypophysitis was found in almost half of the patients. Pituitary disorders preceded the systemic disease in a quarter of the cases. This emphasizes the importance of a systematic repeated workup looking for a secondary etiology of hypophysitis in these patients.