A 41-year-old female patient with a history of sarcoidosis and secondary pulmonary hypertension due to pulmonary embolism presented with headaches and menstrual disturbances. Elevated PRL levels and a hypophyseal macroadenoma was found during the initial workup. Thus, dopamine agonist therapy was initiated, resulting in the normalization of PRL levels. In April, she was admitted to the Korányi National Pulmonology Institute’s intensive care unit due to hypotension, severe weakness, polyuria, and polydipsia, asking an endocrine consultation. During our consultation, we diagnosed central hypoadrenalism and central diabetes insipidus, prompting glucocorticoid supplementation with hydrocortisone and nasal desmopressin therapy. Central hypothyroidism was ruled out. After initiating therapy, the symptoms resolved rapidly. However, during follow-up, complaints of headaches and recurrent fever led to a bronchoscopy, confirming the recurrence of pulmonary sarcoidosis, and warranted the reintroduction of high-dose methylprednisolone therapy. Even though in September the pituitary lesion decreased in size, on the control MRI, it no longer resembled a macroadenoma but rather a granulomatous lesion. Considering the patient’s symptoms, examinations, laboratory results, and MRI findings, we established a diagnosis of neurosarcoidosis localized to the pituitary and hypothalamic region. Treatment with methylprednisolone was continued in collaboration with the pulmonologist.

Sarcoidosis is a disease of unknown etiology characterized by non-caseating granulomas that can affect any organ. Neurosarcoidosis is confirmed in approximately 14–50% of cases through post-mortem examination, but clinical symptoms manifest in only 5–13% of patients. Lesions involving the pituitary and hypothalamus are rare, 89/2024 11 and as of now, no standardized recommendations have been established for their management. These lesions can lead to hypopituitarism, diabetes insipidus, and optic neuropathy. In cases involving the pituitary and hypothalamus, the primary therapeutic goals include hormone replacement and the reduction of visual field deficits.

For patients undergoing treatment for sarcoidosis, if dysfunction of the hypothalamic-pituitary axis is suspected, the possibility of neurosarcoidosis should be considered, prompting urgent investigation and treatment.