Lymphocytic hypophysitis: clinical characteristics and endocrine features of 64 GH deficient patients in KIMS: Pfizer International Metabolic Database
Sophie Bensing1, Peter Jönsson2, Anna-Lena Hulting1, David Cook3, Murray Gordon4, Michael Faust5, Maria Koltowska-Häggström2 & Felipe Casanueva6
Objective: To characterize patients with GH deficiency associated with lymphocytic hypophysitis (LyH).
Method: Patients with a diagnosis of LyH were identified in the KIMS database. The responsible clinicians were asked to confirm the diagnosis and to provide more detailed information on the patient by filling in a questionnaire.
Results: One hundred and fifty patients with a diagnosis of LyH were identified in the database and 100 clinicians responded to our questionnaire. The LyH diagnosis was confirmed in 64 patients (13 histopathological proven and 51 suspected). The majority of the 64 LyH patients were female (n=49, 76%) and the mean age at diagnosis was 41.4 years. One patient was diagnosed with LyH during pregnancy and nine patients during the postpartum period. At diagnosis 58 patients had symptoms of pituitary hormonal deficiency, 25 patients suffered from headaches and 11 had visual disturbances. Low GH and IGF1 levels were detected in the majority of patients already at diagnosis of LyH, often in combination with other pituitary hormonal deficiencies. Radiological examination revealed an enlarged pituitary in 27 patients in some causing chiasmal compression or involvement of cavernous sinus. Regarding treatment of LyH, 13 patients underwent neurosurgical intervention and six received supraphysiological doses of glucocorticoids. One patient with histopathological proven LyH also received azathioprine and radiotherapy due to relapse of the disease. The most common associated autoimmune conditions were Hashimotos thyroiditis (16/64), type 1 diabetes mellitus (6/64) and primary ovarian/testicular failure (5/64).
Conclusion: Deficits of ACTH, TSH and gonadotrophins are commonly reported in LyH. However, to a great extent GH function has not been evaluated. Our survey shows that GH deficiency may be present at the time of diagnosis of LyH, stressing the need for proper assessment of all pituitary hormones in these patients. LyH is frequently associated with other autoimmune conditions.