Diagnosis and Treatment of Autoimmune and IgG4-related Hypophysitis：Clinical Guidelines of the Japan Endocrine Society in 2020：Interpretation and Implication to General Practice Development in China

doi:10.12114/j.issn.1007-9572.2020.00.582

Abstract

Abstract: China issued the Compendium of China's First List of Rare Diseases and 2019 Chinese Guidelines for the Diagnosis and Management of Rare Diseases in 2018 and 2019，which shows that the Chinese government attaches great importance to the diagnosis，treatment and security of rare diseases.Autoimmune hypophysitis is a rare disease that can cause pituitary dysfunction.As low morbidity，changeable symptoms and untypical imaging characteristics，it is easily misdiagnosed，and untimely treatment may lead to life-threatening outcome.In 2020，the Japan Endocrine Society issued the Diagnosis and Treatment of Autoimmune and IgG4-related Hypophysitis：Clinical Guidelines of the Japan Endocrine Society，which are the first clinical guidelines with clear definition，diagnosis and treatment of autoimmune hypophysitis and IgG4-related hypophysitis.This paper interprets the guidelines from the definition，diagnosis and treatment of autoimmune hypophysitis and IgG4-related hypophysitis，and analyzes its implication to general practice development in China，providing a reference for general practitioners' diagnosis and treatment in China.

Key words: Pituitary diseases, Autoimmune diseases, Immunoglobulin G, Guidebooks, Interpretation, General practitioners

摘要： 2018、2019年我国分别颁布了《第一批罕见病目录》《罕见病诊疗指南（2019年版）》，足见我国对罕见病的诊疗和保障工作的重视。自身免疫性垂体炎（AH）属于罕见病，其可导致垂体功能障碍，如不及时救治可危及患者生命，但因其发病率低、症状多变、影像学特点不明显而容易误诊。2020年2月日本内分泌学会（JES）发布了《自身免疫性垂体炎和IgG4相关垂体炎的诊断和治疗指南》（简称JES指南），其是目前国内外首次明确定义AH和IgG4相关垂体炎（IgG4H）的临床诊疗指南。本文从AH和IgG4H的定义、诊断和治疗三个方面对该指南进行解读，并分析了JES指南对我国全科诊疗的启示，旨在为我国全科医生诊疗此类疾病提供参考依据。

关键词: 垂体疾病, 自身免疫疾病, 免疫球蛋白G, 指南, 解读, 全科医生

CHEN Xiaoyi,CHEN Shuchun. Diagnosis and Treatment of Autoimmune and IgG4-related Hypophysitis：Clinical Guidelines of the Japan Endocrine Society in 2020：Interpretation and Implication to General Practice Development in China　[J]. Chinese General Practice, 2020, 23(36): 4535-4540. DOI: 10.12114/j.issn.1007-9572.2020.00.582.
陈晓依,陈树春. 2020年日本内分泌学会《自身免疫性垂体炎和IgG4相关垂体炎的诊断和治疗指南》解析及对我国全科诊疗的启示[J]. 中国全科医学, 2020, 23(36): 4535-4540. DOI: 10.12114/j.issn.1007-9572.2020.00.582.

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