Abstract: Primary immunodeficiency disease is a group of diseases caused by genetic factors and characterized by recurrent，refractory infections. Classification of primary immunodeficiency disease is updated every 2 or 3 years. In 2017，the classification first introduced the term of inborn error of immunity for replacement，but the term "primary immunodeficiency disease" is still in use today. The latest version of the classification has been published by the International Union of Immunological Societies Expert Committee on January 17，2020，including 430 inborn errors of immunity（divided into 10 categories），based on which this article is aimed to interpret the adjustment of the classification.

Key words: primary immunodeficiency disease, classification

摘要： 原发性免疫缺陷病是遗传因素导致的以反复、难治性感染为特征的一组疾病。原发性免疫缺陷病的分类每2～3年更新1次，2017年版分类首次提出“免疫出生错误”这一概念，但目前原发性免疫缺陷病仍在沿用。最新版原发性免疫缺陷病分类已于2020-01-17由国际免疫学会联合会专家委员会发表，包括430种原发性免疫缺陷病，分为10个大类。文章以最新版的原发性免疫缺陷病分类为基础，对原发性免疫缺陷病分类的调整进行解读。

关键词: 原发性免疫缺陷病, 分类