Clinical Characteristics of 76 Patients with IgG4-Related Hypophysitis: A Systematic Literature Review

Yujuan Li,Fengao Li,Xinxin Zhang,Yizhi Ding,Hua Gao,Zhen Li

doi:10.1155/2019/5382640

Yujuan Li, Fengao Li + Show 4 more

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https://doi.org/10.1155/2019/5382640

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Abstract

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.

Highlights

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
IgG4-related disease (IgG4-RD) is a rare, newly recognized, multiorgan involved disease, which was characterized by infiltration of IgG4-positive plasma cells into the organs and elevated serum IgG4 level. e incidence of IgG4-RD was estimated to be 0.28–1.08/100,000 patients in Japan [1]
We summarized case reports of IgG4-related hypophysitis (IgG4-RH) reported in the English articles and provided a detailed analysis of the clinical features of the 76 cases, aiming to get a better understanding of this recently recognized and rare entity

Summary

Introduction

IgG4-related disease (IgG4-RD) is a rare, newly recognized, multiorgan involved disease, which was characterized by infiltration of IgG4-positive plasma cells into the organs and elevated serum IgG4 level. e incidence of IgG4-RD was estimated to be 0.28–1.08/100,000 patients in Japan [1]. Pituitary, which was a rare involved organ, was initially reported in 2004 [3] It accounted for merely 1.5% of systemic cases of IgG4-RD [4]. 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).

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