Characteristics of Patients with Primary and Secondary Hypophysitis: A Single Center Experience

Presentation Number: SAT 416
Date of Presentation: April 1st, 2017

Hande Mefkure Ozkaya*1, Tugce Apaydin2, Fatma Ela Keskin3 and Pinar Kadioglu4
1Cerrahpaşa Medical School, University of İstanbul, istanbul, Turkey, 2Cerrahpasa Medical School, ISTANBUL, Turkey, 3Cerrahpasa Medical School, University of Istanbul, Istanbul, Turkey, 4Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey

Abstract

Purpose: To report the general characteristics of patients with primary and secondary hypophysitis and to evaluate treatment and long-term follow-up results.

Methods: Medical charts of 2596 patients who were followed-up at University of Istanbul, Cerrahpasa Medical School, Endocrinology and Metabolism Outpatient Clinic between 2007-2016 with a diagnosis of pituitary adenoma, empty sella, and hypopituitarism were reviewed retrospectively. A total of 14 patients (9F/5 M) were diagnosed with hypophysitis.

Results: The mean age of patients at diagnosis was 39.14 ±17.7 years. Median follow-up time was 45 (IQR: 13.5-61.2) months. Four out 7 female patients were postmenauposal and three were nulliparous. All but one patient did not have an accompanying autoimmune disease. The most common symptoms and signs were headache (42.9%) and diabetes inspidus (42.9%), followed by hypopituitarism (35.7%). Four out of 14 patients had secondary hypophysitis (3 patients with histiocytosis X, 1 patient with neurosarcoidosis). The remaining 10 patients had primary hypophysitis (8 patients with lymphocytic hypophysitis, 2 patients with xanthomatous hypophysitis). A total of 9 patients were operated. Eight out of 9 patients had a presumptive diagnosis of pituitary macroadenoma. These patients had the histopathological evidence of hypophysitis. One patient was operated due to the mass effect of the lesion .The presumptive diagnosis for this case was lymphocytic hypophysitis which was confirmed by histopathology. Five patients were started on glucocorticoid therapy. The starting doses for metylprednisolone were 60 mg/d in three patients and 120mg/d in two patients. The glucocorticoid dose was tapered in 2 months. The clinical response was assessed in 4 patients . Half of the patients were good responders. Avascular necrosis of femoral head was the most common complication (40%) after glucocorticoid therapy. Two patients with primary hypophysitis required gama-knife or cyber-knife radiosurgery after failure of surgery and glucocorticoid therapy . Another patient with histiocytosis X received gama-knife radiosurgery after operation. The response to radiosurgery was good in all of the patients. No significant side effect was detected. Anterior and posterior pituitary hormone deficiencies were still present at the last follow-up visit in all of the patients who had one or more pituitary hormone deficiencies at the time of diagnosis.

Conclusisons: Hypophysitis, albeit rare, may cause severe pituitary dysfunction which tend to persist during the disease course. Misdiagnosis is frequent. Disease’s pathogenesis might involve mechanisms other than autoimmunity in some patients. A considerable number of patients may require combined therapies which might cause serious side effects.

 

Nothing to Disclose: HMO, TA, FEK, PK