title: A case of IgG4-related multifocal fibrosclerosis complicated by central diabetes insipidus.
abstract: A 55-years-old man was admitted to our hospital with a 6-month history of general fatigue, purulent nasal discharge, polyuria, and polydipsia. Endocrinological findings revealed central diabetes insipidus (CDI) with mild anterior pituitary dysfunction. Imaging studies revealed thickening of the proximal end of the pituitary stalk just below the third ventricle, a mass in the paranasal sinus, and a mass encompassing the abdominal aorta. Histopathology of the mass in the paranasal sinus revealed abundant IgG4-positive plasma cells, and the IgG4 serum level was markedly elevated. Thus, he was diagnosed with IgG4-related multifocal fibrosclerosis. Therapy with prednisolone resulted in complete resolution of clinical symptoms and reduction in size of the masses in the affected organs. However, CDI remained unchanged. This is the first case in which the cause of CDI was IgG4-related multifocal fibrosclerosis. IgG4-related sclerosing disease should be included in the differential diagnosis of thickening of the pituitary stalk with CDI, and a search for extra-pituitary involvement is essential.
authors.affiliation: Department of Internal Medicine, Osaka City General Hospital, Osaka, Japan.
authors.forename: Yoshihiro
authors.initials: Y
authors.lastname: Isaka
authors.forename: Katsunobu
authors.initials: K
authors.lastname: Yoshioka
authors.forename: Minako
authors.initials: M
authors.lastname: Nishio
authors.forename: Keiko
authors.initials: K
authors.lastname: Yamagami
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Konishi
authors.forename: Takeshi
authors.initials: T
authors.lastname: Inoue
authors.forename: Ayako
authors.initials: A
authors.lastname: Hirano
authors.forename: Masayuki
authors.initials: M
authors.lastname: Hosoi
authors.forename: Masahito
authors.initials: M
authors.lastname: Imanishi
mesh_terms: D001012:Aorta, Abdominal / Q000473:pathology*; D003919:Diabetes Insipidus / Q000150:complications*; D005355:Fibrosis; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008875:Middle Aged; D010256:Paranasal Sinuses / Q000473:pathology*; D010902:Pituitary Gland / Q000473:pathology*; D012598:Sclerosis
chemical_list: D007074:Immunoglobulin G