Endocrine Abstracts

Introduction: Hypophysitis is an inflammatory disease of the pituitary that may mimic tumors. Primary hypophysitis has been classified as lymphocytic (LH), granulomatous (GH), and xanthomatous (XH). It has been recently proposed to be an IgG4-related autoimmune disease (serum IgG4 concentration: 135 mg/dl), proven by tissue IgG4 immunostaining.

Case description: A 23-year-old men suffered from typical cluster type headache. Two years after the first symptoms, diabetes insipidus occured. His testosterone level was low with low serum FSH and LH suggesting central hypogonadism, but all the other anterior pituitary hormone levels were normal. Sella MRI scan depicted a 17 mm inhomogenous mass. After the transphenoidal surgery the pituitary tissue was showed by accumulation of foamy cells and xanthomatous epithelioid cells. After stopping the preoperative hydrocortisone therapy the headache returned. The endrocrine work up revealed hypadrenia (morning cortisol: 96 nmol/l and ACTH: 3.38 pmol/l), hypothyroidism (ft4: 10.5 pmol/l), hypogonadism (testosterone: 3.44 nmol/l) with FSH: 3.3 mIU/l and LH:2.8 mIU/l. Hydrocortisone, levothyroxine and testosterone were stepwise reintroduced. During the follow-up we could stop hydrocortisone, levothyroxine, whereas he has permanently required desmopressin and testosterone substitution. Occasionally headache attacks occur and dissappeare with glucocorticoid administration. Two years after the initial diagnosis the hypophysitis was proven to be IgG4-related by his tissue IgG4 immunostaining as well as by his elevated serum IgG4 concentration (815 mg/l).

Conclusion: We describe a case of IgG4-related xanthomatous hypophysitis causing cluster type headache permanenty requiring ddAVP (desmopressine) and testosterone supplementation however, without need for maintenance medication with hydrocortisone and levothyroxin. In periods of headaches the patient requires glucocorticoids supporting the possible autoimmune origin of the disease.