The prevalence of IgG4-related hypophysitis in 170 consecutive patients with hypopituitarism and/or central diabetes insipidus and review of the literature

Abstract

Objective: The prevalence and clinical characteristics of IgG4-related hypophysitis remain unclear due to the limited number of case reports. Therefore, in this study, we screened consecutive outpatients with hypopituitarism and/or diabetes insipidus (DI) to estimate its prevalence. Methods: A total of 170 consecutive outpatients with hypopituitarism and/or central DI were screened at Kobe University Hospital for detecting IgG4-related hypophysitis by pituitary magnetic resonance imaging, measuring serum IgG4 concentrations, assessing the involvement of other organs, and carrying out an immunohistochemical analysis to detect IgG4-positive cell infiltration. Results: Among the screened cases, 116 cases were excluded due to diagnosis of other causes such as tumors and congenital abnormalities. Additionally, 22 cases with isolated ACTH deficiency were analyzed and were found not to meet the criteria of IgG4-related hypophysitis. The remaining 32 cases were screened and seven were diagnosed with IgG4-related hypophysitis, of which three cases were diagnosed by analyzing pituitary specimens. IgG4-related hypophysitis was detected in 30% (seven of 23 patients) of hypophysitis cases and 4% of all hypopituitarism/DI cases. The mean age at the onset of IgG4-related hypophysitis was 61.8G8.8 years, and the serum IgG4 concentration was 191.1G78.3 mg/dl (normal values 5-105 mg/dl and values in IgG4-related disease (RD) R135 mg/dl). Pituitary gland and/or stalk swelling was observed in six patients, and an empty sella was observed in one patient. Multiple co-existing organ involvement was observed in four of the seven patients prior to the onset of IgG4-related hypophysitis. Conclusion: These data suggest that the prevalence of IgG4-related hypophysitis has been underestimated. We should also consider the possibility of the development of hypopituitarism/DI caused by IgG4-related hypophysitis during the clinical course of other IgG4-RDs.

Hypophysitis: a single-center case series

“…Recent work has identified an IgG4-related plasmacytic secondary hypophysitis variant, often in patients with IgG4 systemic disease [3,14]. Relatively few case reports have been published since first descriptions based on clinical suspicion in 2004 [15], and pathological confirmation in 2007 [16], however a recent review suggests that its incidence may be significantly underreported [17].…”

Choroba Mikulicza-Radeckiego z zapaleniem przysadki — nowe zaburzenie związane z IgG4

“…To our knowledge, there have been only 29 such cases reported so far [4]. The first case of IgG4-related hypophysitis was reported in Japan in the year 2000 [5].…”

“…In the review by Bando et al [4] only 14% of the patients solely exhibited hypophysitis, which was diagnosed by histopathology. In 2011 Leporati et al [9] suggested five criteria to diagnose IgG4-related hypophysitis (Table III).…”

Immunoglobulin G4-Related Thyroid Diseases

“…Hashimoto encephalopathy was found to be associated with high serum and intrathecal IgG4 levels in a case report [59]. IgG4-related hypophysitis has been the subject of case reports and a case series [60]. …”