The complement system plays an essential role in both innate and adaptive immune responses. Any dysregulation in this system can disturb normal host defense and alter inflammatory response leading to both infections and autoimmune diseases. The complement system can be activated through three different pathways. Inherited complement deficiencies have been described for all complement components and their regulators. Despite being rare diseases, complement deficiencies are often severe, with a frequent onset during childhood. We provide an overview of clinical disorders related to these disorders and describe current diagnostic strategies required for their comprehensive characterization and management.
Inherited defects in the complement system / Leonardi, Lucia; La Torre, Francesco; Soresina, Annarosa; Federici, Silvia; Cancrini, Caterina; Castagnoli, Riccardo; Cinicola, BIANCA LAURA; Corrente, Stefania; Giardino, Giuliana; Lougaris, Vassilios; Volpi, Stefano; Luigi Marseglia, Gian; Cardinale, Fabio. - In: PEDIATRIC ALLERGY AND IMMUNOLOGY. - ISSN 1399-3038. - (2022).
Inherited defects in the complement system
Lucia Leonardi;Bianca Laura Cinicola;
2022
Abstract
The complement system plays an essential role in both innate and adaptive immune responses. Any dysregulation in this system can disturb normal host defense and alter inflammatory response leading to both infections and autoimmune diseases. The complement system can be activated through three different pathways. Inherited complement deficiencies have been described for all complement components and their regulators. Despite being rare diseases, complement deficiencies are often severe, with a frequent onset during childhood. We provide an overview of clinical disorders related to these disorders and describe current diagnostic strategies required for their comprehensive characterization and management.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.
