Cargando…

Primary Immunodeficiency Diseases: Current and Emerging Therapeutics

Primary immunodeficiency diseases (PID) result from defects in genes affecting the immune and other systems in many and varied ways (1, 2). Until the last few years, treatments have been largely supportive, with the exception of bone marrow transplantation. However, recent advances in immunobiology,...

Descripción completa

Detalles Bibliográficos
Autores principales: Marciano, Beatriz E., Holland, Steven M.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5552668/
https://www.ncbi.nlm.nih.gov/pubmed/28848545
http://dx.doi.org/10.3389/fimmu.2017.00937
_version_ 1783256489903783936
author Marciano, Beatriz E.
Holland, Steven M.
author_facet Marciano, Beatriz E.
Holland, Steven M.
author_sort Marciano, Beatriz E.
collection PubMed
description Primary immunodeficiency diseases (PID) result from defects in genes affecting the immune and other systems in many and varied ways (1, 2). Until the last few years, treatments have been largely supportive, with the exception of bone marrow transplantation. However, recent advances in immunobiology, genetics, and the explosion of discovery and commercialization of biologic modifiers have drastically altered the landscape and opportunities in clinical immunology. Therapeutic options and life expectancy of PID patients have also improved dramatically, in large part as a result of better prevention and treatment of infections as well as better understanding and treatment of autoimmune complications (3). As early-life infection-related mortality declines we should anticipate the emergence of other conditions that were previously not appreciated, including malignancies and degenerative disorders unmasked by increasing longevity (4). The genomic revolution has identified literally hundreds of new genetic etiologies of immune dysfunction, many of which are or will soon be eligible for targeted therapies. These emerging immunomodulatory agents represent new therapeutic options in PIDs (5).
format Online
Article
Text
id pubmed-5552668
institution National Center for Biotechnology Information
language English
publishDate 2017
publisher Frontiers Media S.A.
record_format MEDLINE/PubMed
spelling pubmed-55526682017-08-28 Primary Immunodeficiency Diseases: Current and Emerging Therapeutics Marciano, Beatriz E. Holland, Steven M. Front Immunol Immunology Primary immunodeficiency diseases (PID) result from defects in genes affecting the immune and other systems in many and varied ways (1, 2). Until the last few years, treatments have been largely supportive, with the exception of bone marrow transplantation. However, recent advances in immunobiology, genetics, and the explosion of discovery and commercialization of biologic modifiers have drastically altered the landscape and opportunities in clinical immunology. Therapeutic options and life expectancy of PID patients have also improved dramatically, in large part as a result of better prevention and treatment of infections as well as better understanding and treatment of autoimmune complications (3). As early-life infection-related mortality declines we should anticipate the emergence of other conditions that were previously not appreciated, including malignancies and degenerative disorders unmasked by increasing longevity (4). The genomic revolution has identified literally hundreds of new genetic etiologies of immune dysfunction, many of which are or will soon be eligible for targeted therapies. These emerging immunomodulatory agents represent new therapeutic options in PIDs (5). Frontiers Media S.A. 2017-08-09 /pmc/articles/PMC5552668/ /pubmed/28848545 http://dx.doi.org/10.3389/fimmu.2017.00937 Text en Copyright © 2017 Marciano and Holland. http://creativecommons.org/licenses/by/4.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Immunology
Marciano, Beatriz E.
Holland, Steven M.
Primary Immunodeficiency Diseases: Current and Emerging Therapeutics
title Primary Immunodeficiency Diseases: Current and Emerging Therapeutics
title_full Primary Immunodeficiency Diseases: Current and Emerging Therapeutics
title_fullStr Primary Immunodeficiency Diseases: Current and Emerging Therapeutics
title_full_unstemmed Primary Immunodeficiency Diseases: Current and Emerging Therapeutics
title_short Primary Immunodeficiency Diseases: Current and Emerging Therapeutics
title_sort primary immunodeficiency diseases: current and emerging therapeutics
topic Immunology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5552668/
https://www.ncbi.nlm.nih.gov/pubmed/28848545
http://dx.doi.org/10.3389/fimmu.2017.00937
work_keys_str_mv AT marcianobeatrize primaryimmunodeficiencydiseasescurrentandemergingtherapeutics
AT hollandstevenm primaryimmunodeficiencydiseasescurrentandemergingtherapeutics