Endocrine Abstracts

Introduction: Hypothalamic-pituitary involvement during sarcoidosis is rare, less than 1%. Granulomatous infiltration can lead to anteropituitary insufficiency, disconnection hyperprolactinemia and diabetes insipidus. We report a case of hypothalamic-pituitary sarcoidosis with review of the literature.

Observation: Patient aged 39 years, she complains of chronic headaches for 10 months without decrease in visual acuity, secondary amenorrhea for 6 months. The clinical examination reveals a bilateral multiporous induced galactorrhea. The hypothalamo-hypophyseal MRI showed a thickening of the pituitary stem. The hormonal assessment revealed a cortisol level of 3.6 mg/dl at 8 o’clock in the morning, prolactin at 64.6 ng/ml, FSH at 0.7 mIU/ml, LH at 0.6 mIU/ml, estradiol: 5ng/l, TSH us at 2.08 uIU/ml, free thyroxine (T4L) at 12.7 pmol/l, corrected calcemia at 89.49 mg/l, the visual field showed no abnormality. The general work-up revealed a pulmonary localization with a stage II sarcoidosis on the thoracic CT scan and a skin involvement confirmed on biopsy: an aspect of epithelioid and giganto-cellular granulomatous hypodermitis without caseous necrosis. The patient was put on corticosteroid therapy and hormone replacement therapy with estrogen-progestin

Discussion: Sarcoidosis is a chronic systemic disease of unknown etiology characterized by the presence of epithelioid granuloma without caseous necrosis. Neurosarcoidosis is found in 5-26% of patients with sarcoidosis. Isolated hypothalamic-pituitary involvement is rare, representing 0.5% of sarcoidosis cases and 1% of sella turcica lesions. The endocrine disorders most frequently encountered in neurosarcoidosis are: diabetes insipidus, hyperprolactinemia and to a lesser degree hypogonadism. However, a review of the literature published by Anthony et al classified the endocrine disorders in order of frequency: central hypogonadism in 88.8% of cases, central hypothyroidism in 67.4% of cases, diabetes insipidus in 65.2% of cases, low GH levels in 54% of cases, low ACTH levels in 48.8% of cases, hyperprolactinemia in 48.8% of cases. The most common radiological abnormalities in neurosarcoidosis are multiple white matter involvement (43%) and pathological contrast of the meninges (38%), rarely obstructive hydrocephalus or spinal cord involvement. Imaging may be normal in 12% of cases. The treatment is mainly medical with administration of corticosteroids; in case of poor response to corticosteroids, other therapies may be prescribed such as immonosuppressive drugs and immunomodulators; radiotherapy may also be indicated in some refractory cases.

Conclusion: Hypothalamic-pituitary involvement in sarcoidosis is rare with more frequent complications, the diagnosis is often late. It requires a multidisciplinary management.