Immunoglobulin G4 hypophysitis in a 63-year-old woman with no autoimmune history: a case report

Abstract

Background Immunoglobulin-G4-related hypophysitis is a rare inflammatory disease that can present as a tumefactive pituitary lesion mimicking hypophyseal neoplasms such as pituitary adenoma or craniopharyngioma. The literature on this entity is sparse, with fewer than 100 cases reported across 19 publications; a recent review found only 24 cases published from 2007 to 2018. Previous reports have described demographic differences, with immunoglobulin-G4-related hypophysitis in females tending to present in the second and third decades in association with other autoimmune disease, while males tend to present in the fifth and sixth decades of life without an autoimmune history. Case presentation In contrast to the reported demographic trends, here we describe a unique case of immunoglobulin-G4-related hypophysitis in a 63-year-old white female with no history of autoimmune disease who presented with a rapidly enlarging sellar and hypothalamic mass causing headaches and cranial nerve palsies, prompting biopsy for diagnosis. The patient experienced rapid response to treatment with high-dose steroids and rituximab. Conclusion The case contributes to the growing clinicopathologic description of immunoglobulin-G4-related hypophysitis and illustrates that this diagnosis should be a consideration even outside the conventional demographic setting.

Spectre de manifestations de la maladie liée à l’IgG4 : une revue de la littérature

“…La recherche dans les bases de données mentionnées s'est faite par la combinaison ou l'utilisation isolée de ces descripteurs. Hypophysite liée aux DR-IgG4 Alrashdi, 2020;Al-Mujaini et al, 2018;Bong et al, 2021;Boban, Ardal e Thurnher, 2018;Gersey et al, 2021;Ota et al ,2020;Levraut et al, 2019.…”

Espectro de manifestaciones de la enfermedad relacionada con IgG4: una revisión de la literatura

“…Paquimeningitis espinal por DR-IgG4 Paquimeningitis hipertrófica con higroma subdural poliquístico y hematoma en DR-IgG4 Hipofisitis relacionada con DR-IgG4 Alrashdi, 2020;Al-Mujaini et al, 2018;Bong et al, 2021;Boban, Ardal y Thurnher, 2018;Gersey et al, 2021;Ota et al, 2020;Levraut et al, 2019.…”

Glucocorticoid impact therapy for recurrent IgG4-related disease with diabetes insipidus as the main manifestation: A case report and literature review

“…We used "hypophysitis," "IgG4-related hypophysitis," "diabetes insipidus" as keywords and searched on PubMed database. The review literature has been available for 115 cases of IgG4-RH before 2021, [1] so the search period was from January 1, 2021 to December 1, 2022, and 15 case reports were detected, [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] including 12 with complete clinical information and a total of 24 patients [2][3][4][5][6][7][9][10][11][12][13][14] (Table 1).…”