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

Journal of Medical Case Reports

Scott A. Segel¹  Kenan R. Rajjoub²  Paul A. Gardner³  Zachary C. Gersey³  Georgios A. Zenonos³  Carl H. Snyderman⁴  Eric W. Wang⁴  Thomas M. Pearce⁵ 

[1] Department of Endocrinology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA;Department of Neurological Surgery, The George Washington University School of Medicine, Washington, District of Columbia, USA;Department of Neurological Surgery, University of Pittsburgh Medical Center, 200 Lothrop St Suite B-400, 15213, Pittsburgh, PA, USA;Department of Otolaryngology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA;Division of Anatomic Pathology, Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, PA, USA;

关键词: IgG4;    Hypophysitis;    Endonasal;    Skull base;    Case report;   

DOI  :  10.1186/s13256-021-03018-7

来源: Springer

【 摘 要 】

BackgroundImmunoglobulin-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 presentationIn 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.ConclusionThe 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.

【 授权许可】

CC BY