Cordero, ElisaGoycochea Valdivia, WalterMendez Echevarría, AnaM. Allende, LuisAlsina, LaiaBravo García Morato, MaríaGil Herrera, JuanaGudiol, CarlotaLen Abad, ÓscarLópez Medrano, FranciscoMoreno Pérez, DavidMuñoz, PatriciaOlbrich, PeterSánchez Ramón, Silvia MaríaSoler Paracín, PereAguilera Cros, ClaraArostegui, Juan IgnacioBadell Serra, IsabelCarbone, JavierFortún, JesúsGonzález Granado, Luis IgnacioLópez Granados, EduardoLucena, José ManuelParody, RocíoRamakers, JanRegueiro González-Barros, José RamónRivière, Jacque GRoca Oporto, CristinaRodríguez Pena, RebecaSantos Pérez, Juan LuisRodríguez Gallego, CarlosNeth, Olaf2023-06-172023-06-172020-122213-219810.1016/j.jaip.2020.05.008https://hdl.handle.net/20.500.14352/8576Primary immunodeficiencies (PIDs) are rare, undiagnosed and potentially fatal diseases. Clinical manifestations of PID can be fatal or leave sequelae that worsen the quality of life of patients. Traditionally, the treatment of PIDs has been largely supportive, with the exception of bone marrow transplantation and, more recently, gene therapy. The discovering of new affected pathways, the development of new molecules and biologics, and the increasing understanding of the molecular basis of these disorders have created opportunities in PIDs therapy. This document aims to review current knowledge and to provide recommendations about the diagnosis and clinical management of adults and childre with PIDs based on the available scientific evidence taking in to account current practice and future challenges. A systematic review was conducted, and evidence levels based on the available literature are given for each recommendation where available. � 2020 American Academy of Allergy, Asthma & Immunology; Sociedad Española de Enfermedades Infecciosas y Microbiología Clínica [Published by Elsevier Inc./Elsevier Espana]. All rights reserved.engjournal articlehttps://doi.org/10.1016/j.jaip.2020.05.008restricted accessPrimary immunodeficienciesVaccinationTreatmentHaematopoietic progenitors transplantationAntibioticsInmunologíaOncología2412 Inmunología3201.01 Oncología