Bousfiha Aziz, Jeddane Leïla, Al-Herz Waleed, Ailal Fatima, Casanova Jean-Laurent, Chatila Talal, Conley Mary Ellen, Cunningham-Rundles Charlotte, Etzioni Amos, Franco Jose Luis, Gaspar H Bobby, Holland Steven M, Klein Christoph, Nonoyama Shigeaki, Ochs Hans D, Oksenhendler Eric, Picard Capucine, Puck Jennifer M, Sullivan Kathleen E, Tang Mimi L K
Clinical Immunology Unit, A. Harouchi Hospital, Ibn Roshd Medical School, King Hassan II University, Casablanca, Morocco.
Department of Pediatrics, Faculty of Medicine Kuwait University, Jabriya, Kuwait.
J Clin Immunol. 2015 Nov;35(8):727-38. doi: 10.1007/s10875-015-0198-5. Epub 2015 Oct 7.
There are now nearly 300 single-gene inborn errors of immunity underlying phenotypes as diverse as infection, malignancy, allergy, auto-immunity, and auto-inflammation. For each of these five categories, a growing variety of phenotypes are ascribed to Primary Immunodeficiency Diseases (PID), making PIDs a rapidly expanding field of medicine. The International Union of Immunological Societies (IUIS) PID expert committee (EC) has published every other year a classification of these disorders into tables, defined by shared pathogenesis and/or clinical consequences. In 2013, the IUIS committee also proposed a more user-friendly, phenotypic classification, based on the selection of key phenotypes at the bedside. We herein propose the revised figures, based on the accompanying 2015 IUIS PID EC classification.
目前,有近300种单基因遗传性免疫缺陷,其潜在表型多种多样,包括感染、恶性肿瘤、过敏、自身免疫和自身炎症。在这五个类别中,越来越多的表型被归因于原发性免疫缺陷疾病(PID),这使得PID成为医学领域中一个迅速发展的领域。国际免疫学会联盟(IUIS)PID专家委员会(EC)每隔一年发布一次这些疾病的分类表,这些分类是根据共同的发病机制和/或临床后果来定义的。2013年,IUIS委员会还基于床边关键表型的选择,提出了一种更便于用户使用的表型分类方法。在此,我们根据随附的2015年IUIS PID EC分类法提出修订后的数字。