Background: IgG4-related hypophysitis (IgG4-RH) is a rare disease, and its prevalence remains unclear. In recent years, an increasing number of cases have been reported because of the increasing recognition of this disease. We aimed to summarize case reports of IgG4-RH and outline the clinical features and outcomes.
Methods: We performed PubMed search of articles using the search terms "hypophysitis [AND] IgG4." Consequently, only 54 English articles (76 cases) met Leporati's diagnostic criteria.
Results: Of the 76 cases, the ratio of men to women was 1.5 : 1, and the age at diagnosis was 54.1 ± 17.8 years. The median IgG4 concentration was 405.0 mg/dl. Anterior hypopituitarism, isolated central diabetes insipidus, and panhypopituitarism were observed in 14 (18.4%), 12 (15.8%), and 44 (57.9%) cases, respectively. The sequence of anterior hormone deficiency was as follows: gonadotropin (68.4%), ACTH (63.2%), TSH (59.2%), GH (48.7%), and prolactin (42.1%). The median number of involved organs was 1.5, and the lung (18.4%), retroperitoneum (17.1%), kidney (15.8%), submandibular glands (14.5%), and pancreas (13.2%) were the common involved organs. Elevated IgG4 concentration and normal IgG4 level were in 42 (76.4%) and 13 (23.6%) cases, respectively. Patients with elevated serum IgG4 concentration were older (60.9 ± 14.3 vs 45.6 ± 17.4, p=0.001) and male-prone (78.6% vs 40.4%, p=0.003) and had a susceptibility of multiple organ involvement (78.6% vs 35.0%, p=0.001) compared to those with normal serum IgG4 levels. Males were older at disease onset (61.5 ± 12.6 vs 42.9 ± 18.8, p < 0.001) and had a higher IgG4 concentration (425.0 vs 152.5, p=0.029) and a greater number of involved organs (2.0 vs 0.0, p=0.001), while isolated hypophysitis was more prominent in female (63.3% vs 26.1%, p=0.001).
Conclusion: In this review, we found that there were different characteristics between different genders. Patients with elevated serum IgG4 level in terms of some clinical features were also different from those with normal serum IgG4 level. However, the data in this review were limited by bias and confounding. Further clinical studies with larger sample sizes are warranted.
Copyright © 2019 Yujuan Li et al.
Conflict of interest statement
The authors declare that they have no conflicts of interest in this paper.
Critical Review of IgG4-related HypophysitisJ Shikuma et al. Pituitary 20 (2), 282-291. PMID 27812776. - ReviewIgG4-related hypophysitis may have different clinical characteristics between genders. This survey may lack some information because the Japanese abstracts did not contai …
The Prevalence of IgG4-related Hypophysitis in 170 Consecutive Patients With Hypopituitarism and/or Central Diabetes Insipidus and Review of the LiteratureH Bando et al. Eur J Endocrinol 170 (2), 161-72. PMID 24165017. - ReviewThese data suggest that the prevalence of IgG4-related hypophysitis has been underestimated. We should also consider the possibility of the development of hypopituitarism …
Histologically Confirmed Isolated IgG4-related Hypophysitis: Two Case Reports in Young WomenGA Sosa et al. Endocrinol Diabetes Metab Case Rep 2014, 140062. PMID 25298883.IgG4-related hypophysitis belongs to the group of IgG4-related diseases, and is a fibro-inflammatory condition characterized by dense lymphoplasmacytic infiltrates rich i …
Disease Heterogeneity in IgG4-related Hypophysitis: Report of Two Histopathologically Proven Cases and Review of the LiteratureS Uccella et al. Virchows Arch 475 (3), 373-381. PMID 30911814. - ReviewIgG4-related hypophysitis (IgG4-RH) is a rare disease, which can occur singularly or as manifestation of a systemic IgG4-related disease (IgG4-RD). Less than one hundred …
A Case Series of Atypical Features of Patients With Biopsy-Proven Isolated IgG4-related Hypophysitis and Normal Serum IgG4 LevelsKCJ Yuen et al. Pituitary 21 (3), 238-246. PMID 29248982.Our cases highlight the importance of considering IgG4-related hypophysitis in the differential diagnosis of solid and cystic sellar lesions presenting acutely with pitui …