Clinical and hormonal characteristics of patients with different types of hypophysitis: a single-center experience

Abstract

Objective: The inflammation of the pituitary gland is known as hypophysitis. It is a rare disease accounting for approximately 0.24%-0.88% of all pituitary diseases. The natural course of hypophysitis is variable. Main forms are histologically classified as lymphocytic, granulomatous, IgG4 related and xanthomatous. We aim to present our patients with hypophysitis and compare clinical, laboratory and radiological features. Subjects and methods: We retrospectively reviewed our database of 1.293 patients diagnosed with pituitary diseases between 2010 and 2017. Twelve patients with hypophysitis were identified. Demographical data, clinical features, endocrinological dysfunction, imaging findings, treatment courses and follow-up periods were evaluated. Results: The frequency of hypophysitis was found 0.93% in all cases of the pituitary disease. Twelve patients (nine females and three males), ages between 17-61 years, were evaluated. The characteristic features of our patients tended to be predominantly female and young. Diagnosis of hypophysitis was made after pituitary biopsy in four patients, and in eight patients after pituitary operation due to adenoma. Headache (67%) and visual problems (33%) were the most frequent nonendocrine symptoms. Anterior pituitary hormone deficiencies (63.7%) and/or diabetes insipidus (17%) were seen among patients. According to histopathological forms, four had lymphocytic, seven had granulomatous and one had xanthogranulomatous types. Contrast enhancement heterogeneous and thickened pituitary stalk were the most common radiological alterations. Conclusion: Hypophysitis should be considered in the differential diagnosis of sellar masses. It can mimic pituitary adenomas in radiological and endocrinological aspects. The different patterns of pituitary hormone deficiencies may be seen in the course of the disease.

Xanthomatous Hypophysitis: A Case Report and Comprehensive Literature Review

“…There were 36 cases of XHP, including 35 in previous literature (3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22) and one in our center (summarized in Table 2). The mean age at diagnosis was 39.1 years (range 12-72), and 27 (75.0%) patients were female.…”

Hypophysitis (Including IgG4 and Immunotherapy)

“…Hypophysitis is relatively rare, accounting for approximately 0.24–0.93% of all pituitary diseases [4, 8] – with its incidence constantly increasing with the widespread use of ICIs – and 0.4% of pituitary surgical cases [9]. PH is the commonest form of autoimmune hypophysitis [7].…”

Hypophysitis, the Growing Spectrum of a Rare Pituitary Disease

“…Histological classification includes lymphocytic, granulomatous, xanthomatous ( 9 ), IgG-4 related, necrotizing hypophysitis, and mixed forms (lymphogranulomatous, xanthogranulomatous) ( 4 , 10 ); LHy is overall the most common form ( 11 , 12 ). Accurate classification is sometimes not possible due to pathological features overlap ( 4 , 10 , 13-23 ) ( Fig.…”

“…Classically, hypophysitis presents with symptoms related to pituitary deficiencies with or without headaches and vision changes related to the mass effect of an enlarged pituitary gland and infundibulum. Approximately half of patients with primary hypophysitis present with headaches, while 10% to 30% present with visual disturbances related to mass effect ( Table 2 ) ( 12 , 24-40 ).…”

“…Primary hypophysitis has very variable GC response rate, from 20% to >95% in a partial or complete hormonal and radiographic response ( 12 , 24-40 ). Overall improvement in endocrine function occurs less than reduction in pituitary mass, which can attain closer to a 75% response rate ( 11 , 12 , 24-40 ) ( Table 2 ). Earlier GC initiation has been shown to improve hormonal recovery ( 40 ).…”