Introduction on primary immunodeficiency diseases

N. Rezaei, F.A. Bonilla, E. de Vries, K.E. Sullivan, J. Orange, M. Seppänen, Ahmed Aziz Bousfiha, Jennifer Puck

Research output: Chapter in Book/Report/Conference proceedingChapterScientificpeer-review


Primary immunodeficiency diseases (PIDs) are a heterogeneous group of disorders with defects in one or more components of the immune system. Primary immunodeficiency diseases could suffer from a variety of clinical manifestations, including recurrent, severe or unusual infections, autoimmunity, and lymphoproliferation/malignancies. Primary immunodeficiencies constitute a large group of diseases, affecting the development of the immune system, its function, or both. The number of known PIDs has been increased considerably over the last decade, while more than 300 different types have already been explained. Although the number of patients diagnosed with PIDs is growing, health care workers still know little about these disorders. Thus, many patients are diagnosed late; many cases suffer from complications by chronic infections, irretrievable end-organ damage, or even death before the definitive diagnosis is made. Timely diagnosis and appropriate treatment remain the keys to the successful management of patients with PIDs.
Keywords: Primary immunodeficiency diseases, Infections, Autoimmunity, Malignancies
Original languageEnglish
Title of host publicationPrimary immunodeficiency diseases
Subtitle of host publicationDefinition, diagnosis, and management
EditorsN. Rezaei, A. Aghamohammadi, L.D. Notarangelo
PublisherSpringer Verlag
ISBN (Print) 978-3-662-52907-2
Publication statusPublished - 2017


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