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,...
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doaj-f460715c2d65479589f594e370997be72020-11-25T00:40:17ZengFrontiers Media S.A.Frontiers in Immunology1664-32242017-08-01810.3389/fimmu.2017.00937279952Primary Immunodeficiency Diseases: Current and Emerging TherapeuticsBeatriz E. Marciano0Steven M. Holland1Laboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, MD, United StatesLaboratory of Clinical Infectious Diseases, National Institute of Allergy and Infectious Diseases (NIAID), National Institutes of Health (NIH), Bethesda, MD, United StatesPrimary 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).http://journal.frontiersin.org/article/10.3389/fimmu.2017.00937/fullimmunodeficiencyimmune modulationchronic granulomatous diseaseleukocyte adhesion deficiencyinterferon gamma |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Beatriz E. Marciano Steven M. Holland |
spellingShingle |
Beatriz E. Marciano Steven M. Holland Primary Immunodeficiency Diseases: Current and Emerging Therapeutics Frontiers in Immunology immunodeficiency immune modulation chronic granulomatous disease leukocyte adhesion deficiency interferon gamma |
author_facet |
Beatriz E. Marciano Steven M. Holland |
author_sort |
Beatriz E. Marciano |
title |
Primary Immunodeficiency Diseases: Current and Emerging Therapeutics |
title_short |
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_sort |
primary immunodeficiency diseases: current and emerging therapeutics |
publisher |
Frontiers Media S.A. |
series |
Frontiers in Immunology |
issn |
1664-3224 |
publishDate |
2017-08-01 |
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). |
topic |
immunodeficiency immune modulation chronic granulomatous disease leukocyte adhesion deficiency interferon gamma |
url |
http://journal.frontiersin.org/article/10.3389/fimmu.2017.00937/full |
work_keys_str_mv |
AT beatrizemarciano primaryimmunodeficiencydiseasescurrentandemergingtherapeutics AT stevenmholland primaryimmunodeficiencydiseasescurrentandemergingtherapeutics |
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