Endocrine Abstracts

Background and aim: Autoimmune hypophysitis (AH) is a rare immune-mediated inflammatory disease. Glucocorticoid therapy represents a therapeutic choice for AH patients with symptoms from mass effect, such as headache and optic nerve compression. However, effective methods and adverse events of glucocorticoid therapy are unclear. We aimed to evaluate the efficacy and safety of glucocorticoid therapy in AH patients.

Patients and Methods: The subjects were all patients who had been diagnosed as AH and who underwent glucocorticoid therapy at our hospital from January 1, 2013 to December 31, 2018. We performed a retrospective analysis based on the clinical records of these subjects. We investigated the symptoms, endocrine function and treatment complications.

Results: Sixteen patients (median age at diagnosis: 42 years; range, 29–75 years) were included. The initial treatments were; prednisolone (n = 7) methylprednisolone pulse therapy (n = 8) and hydrocortisone (n = 1). The median duration of glucocorticoid treatment was 22 months. The median follow-up was 47.5 months. Headache and visual impairment improved in all patients within 3 months after the initiation of glucocorticoid therapy. Glucocorticoid therapy was effective for achieving mass reduction on MRI in 14 cases (88%). However, four patients relapsed during treatment with physiologic dose of hydrocortisone. Two of these patients underwent biopsy of the enlarged lesion again. In these cases, the diagnosis changed to germinoma and malignant lymphoma, respectively. As for the endocrine function, the change in the number of cases of hypopituitarism after the initiation of glucocorticoid therapy was changed as follows: GH, 12 to 7 cases; PRL, 8 to 2; gonadotropin, 10 to 7; TSH, 10 to 11; ACTH, 9 to 12; ADH, 12 to 11. As for adverse events, the mean BMI significantly increased from 24.6 to 27.0 kg/m². Two patients newly developed diabetes mellitus and one patient developed femoral head necrosis. These adverse events appeared in patients who underwent pulse therapy followed by prednisolone.

Discussion: Our study revealed that glucocorticoid therapy contributed to the improvement of symptoms due to mass effect in AH patients but did not improve their endocrine functions. It must be noted that two of recurrent cases were insidious malignant diseases. It is important to carefully reassess the diagnosis and clinical course without blindly believing pathological data. In addition, the combination of steroid pulse therapy and prednisolone caused some complications but also ameliorated diabetes insipidus in one case. Further studies are required to establish the efficacy and safety of glucocorticoid therapy in AH patients.