Endocrine Abstracts

Introduction: Idiopathic granulomatous hypophysitis (IGH) is a rare inflammatory disease that may present with radiological changes in the pituitary gland. Diagnosis with non-specific syndromes becomes challenging. Secondary causes of granulomatous hypophysitis include tuberculosis, sarcoidosis, syphilis, Langerhans cell histiocytosis, GPA, and Rathke’s cleft cyst rupture. GPA is a multisystem disease, characterized by necrotizing small-vessel vasculitis, mostly affecting the lung, kidneys, ears, nose, and throat, but potentially it can involve any organs. Pituitary involvement in GPA is rare, estimated occurrence is about 1 % of all cases.

Case description: The history of the 18-year-old woman began a few months before admitting to our Department, with a throbbing headache and dropping the right eyelid. Her CT revealed no pathology, however in head MR the sellar mass (12x14x14mm) was described. Additionally, mucosal changes filling almost completely the left maxillary sinus and the ethmoid on the left side were found. The lesion was described as craniopharyngioma. Four months later pituitary MR demonstrated a sellar lesion (17×21×11 mm) with compression of the optic chiasm. The pituitary gland could not be delineated in the sellar region. Mucosal thickening was observed in all sinuses. It has been described again as intrasellar and suprasellar mass which might correspond to a craniopharyngioma. Additional tests revealed high antinuclear antibodies ANA titer, positive anti-RNP/Sm antibodies, and antibodies against B centromeres and DFS 70. Due to the positive result of the Schrimer test, the suspicion of Sjögren’s syndrome was raised. During the next month, the patient’s clinical status deteriorated. She reported fever, fatigue, and headache, along with nausea and vomiting, occurring mostly in the morning. She underwent transsphenoidal resection of the pituitary region mass. After the surgery the pituitary hormonal assessment was done, confirming panhypopituitarism. Despite the surgical procedure and the hormonal replacement therapy, the patient’s general condition did not improve as it was expected. The obtained pathomorphological results indicated granulomatous inflammation. Once the histopathologic diagnosis was established, the patient underwent further tests for systemic granulomatous disease. GPA was diagnosed based on recurrent sinusitis, and positive autoantibodies c-ANCA.

Conclusion: The correct diagnosis is essential to avoid surgical treatment, which is not recommended in pituitary GPA. Publications present satisfactory responses to steroid and immunosuppressive treatment. It is important to keep in mind the rare causes of damage of the sellar region and not neglect of coexisting symptoms even those not specific to pituitary diseases.