Critical review of IgG4-related hypophysitis.
OBJECTIVE
IgG4-related hypophysitis is a rare disease, with only 34 cases published in English (2015). Available short reviews may not present complete details of IgG4-related hypophysitis. We aimed to survey case reports of IgG4-related hypophysitis, including abstracts of scientific meetings, in English and Japanese.


METHODS
We searched for information about IgG4-related hypophysitis in PubMed and Igakuchuozasshi (Japan Medical Abstracts Society). Among 104 case reports found, we reviewed 84 fulfilling Leporati's diagnostic criteria.


RESULTS
The mean ±  SD age of onset was 64.2  ±  13.9, 67.5  ±  9.8, and 56.4  ±  18.6 years for all subjects, men, and women, respectively. Men:women was 2.4:1. On magnetic resonance imaging, pituitary, stalk, and pituitary-stalk mass were observed at frequencies of 14.3, 21.4, and 64.3%, respectively. Manifestations were anterior hypopituitarism in 26.2% (22 cases), central diabetes insipidus in 17.9% (15 cases), and panhypopituitarism in 52.4% (44 cases). The median level of serum IgG4 was 264.5 mg/dL for all subjects, 405 mg/dL for men, and 226 mg/dL for women. The mean number of IgG4-related systemic diseases was 2.7  ±  1.5 in all subjects, 3.0  ±  1.5 in men, and 1.8  ±  1.1 in women. Among the IgG4-related diseases, retroperitoneal fibrosis was the most frequent (26.2%), followed by salivary gland diseases (25%). Glucocorticoid therapy was generally effective, except for two cases that received replacement doses. There were significant differences between sexes in terms of age, serum IgG4 levels, and number of IgG4-related diseases.


CONCLUSIONS
IgG4-related hypophysitis may have different clinical characteristics between genders. This survey may lack some information because the Japanese abstracts did not contain certain details.
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Junpei
J
Shikuma
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Kenshi
K
Kan
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Rokuro
R
Ito
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Kazuo
K
Hara
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Hiroyuki
H
Sakai
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Takashi
T
Miwa
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan.
Akira
A
Kanazawa
Department of Diabetology, Metabolism, and Endocrinology, Tokyo Medical University Hospital, 6-7-1 Nishishinjuku, Shinjuku-ku, Tokyo, 160-0023, Japan. odawara@tokyo-med.ac.jp.
Masato
M
Odawara
D000368:Aged; D000069281:Autoimmune Hypophysitis / Q000097:blood* / Q000175:diagnosis* / Q000188:drug therapy; D003919:Diabetes Insipidus / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007018:Hypopituitarism / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy; D007074:Immunoglobulin G / Q000097:blood; D008297:Male; D008875:Middle Aged; D012185:Retroperitoneal Fibrosis / Q000097:blood / Q000175:diagnosis / Q000188:drug therapy
D005938:Glucocorticoids; D007074:Immunoglobulin G
Diabetes insipidus; Glucocorticoid; Hypopituitarism; IgG4-related hypophysitis; Pituitary