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Pulmonary Manifestations of Primary Immunodeficiency Diseases pp 213–235Cite as

Pulmonary Manifestations of Complement Deficiencies

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Abstract

The complement system has an important role in host defense against infections, removal of apoptotic cells and immune complexes, and modulating the adaptive immune response. Deficiencies of the proteins or regulators of the complement system contribute to infections affecting the respiratory tract. The main etiological agents associated with complement deficiencies are encapsulated bacteria as Streptococcus, Haemophilus, and Neisseria, related to the role of this system to bacteria opsonization and clearance. MBL deficiency contributes to infectious risk in some diseases like cystic fibrosis and common variable immunodeficiency (CVID), whose pulmonary impairment is relevant. Many complement deficiencies do not have a severe phenotype; thus, there is restricted information about long-term effects and specific infections such as pulmonary infections. Immunization against encapsulated bacteria is recommended for all these defects.

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Authors and Affiliations

  1. Center of Reference for Rare Diseases, Clinical Immunology, Faculdade de Medicina ABC, Santo Andre, SP, Brazil

    Anete Sevciovic Grumach

  2. ARC 1216 Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Kathleen E. Sullivan

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  1. Anete Sevciovic Grumach
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  2. Kathleen E. Sullivan
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  1. Pediatric Respiratory Diseases Research Center, National Research Institute of Tuberculosis and Lung Diseases (NRITLD), Shahid Beheshti University of Medical Sciences, Tehran, Iran

    Seyed Alireza Mahdaviani

  2. Research Center for Immunodeficiencies Children’s Medical Center, Tehran University of Medical Sciences (TUMS), Tehran, Iran

    Nima Rezaei

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Grumach, A.S., Sullivan, K.E. (2019). Pulmonary Manifestations of Complement Deficiencies. In: Mahdaviani, S., Rezaei, N. (eds) Pulmonary Manifestations of Primary Immunodeficiency Diseases . Springer, Cham. https://doi.org/10.1007/978-3-030-00880-2_8

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