{Reference Type}: Journal Article
{Title}: Critical review of IgG4-related hypophysitis.
{Author}: Shikuma J;Kan K;Ito R;Hara K;Sakai H;Miwa T;Kanazawa A;Odawara M;
{Journal}: Pituitary
{Volume}: 20
{Issue}: 2
{Year}: Apr 2017
{Factor}: 3.599
{DOI}: 10.1007/s11102-016-0773-7
{Abstract}: 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.