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The autoimmune conundrum in common variable immunodeficiency disorders

The autoimmune conundrum in common variable immunodeficiency disorders Downloaded from http://journals.lww.com/co-allergy by BhDMf5ePHKbH4TTImqenVA5KvPVPZ0P5BEgU+IUTEfzO/GUWifn2IfwcEVVH9SSn on 06/02/2020 REVIEW URRENT The autoimmune conundrum in common variable PINION immunodeficiency disorders a b Annick A.J.M. van de Ven and Klaus Warnatz Purpose of review Autoimmune and inflammatory manifestations are the biggest clinical challenge in the care of patients with common variable immunodeficiency (CVID). The increasing pathogenic knowledge and potential therapeutic implications require a new evaluation of the status quo. Recent findings The conundrum of the simultaneous manifestation of primary immunodeficiency and autoimmune disease (AID) is increasingly elucidated by newly discovered genetic defects. Thus, cytotoxic T lymphocyte-associated antigen 4 or caspase-9 deficiency presenting with CVID-like phenotypes reiterate concepts of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome and autoimmune lymphoproliferative syndrome. Activating signaling defects downstream of antigen or cytokine receptors are often associated with loss-of-tolerance in the affected patients. Increasingly, forms of combined immunodeficiency are discovered among CVID-like patients. Although different autoimmune manifestations often coincide in the same patient their immunopathology varies. Treatment of AID in CVID remains a challenge, but based on a better definition of the immunopathology first attempts of targeted treatment have been made. Summary The increasing comprehension of immunological concepts promoting AID in CVID will allow better and in some cases http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Current Opinion in Allergy & Clinical Immunology Wolters Kluwer Health

The autoimmune conundrum in common variable immunodeficiency disorders

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References (117)

Publisher
Wolters Kluwer Health
ISSN
1528-4050
eISSN
1473-6322
DOI
10.1097/ACI.0000000000000218
Publisher site
See Article on Publisher Site

Abstract

Downloaded from http://journals.lww.com/co-allergy by BhDMf5ePHKbH4TTImqenVA5KvPVPZ0P5BEgU+IUTEfzO/GUWifn2IfwcEVVH9SSn on 06/02/2020 REVIEW URRENT The autoimmune conundrum in common variable PINION immunodeficiency disorders a b Annick A.J.M. van de Ven and Klaus Warnatz Purpose of review Autoimmune and inflammatory manifestations are the biggest clinical challenge in the care of patients with common variable immunodeficiency (CVID). The increasing pathogenic knowledge and potential therapeutic implications require a new evaluation of the status quo. Recent findings The conundrum of the simultaneous manifestation of primary immunodeficiency and autoimmune disease (AID) is increasingly elucidated by newly discovered genetic defects. Thus, cytotoxic T lymphocyte-associated antigen 4 or caspase-9 deficiency presenting with CVID-like phenotypes reiterate concepts of immune dysregulation, polyendocrinopathy, enteropathy, X-linked syndrome and autoimmune lymphoproliferative syndrome. Activating signaling defects downstream of antigen or cytokine receptors are often associated with loss-of-tolerance in the affected patients. Increasingly, forms of combined immunodeficiency are discovered among CVID-like patients. Although different autoimmune manifestations often coincide in the same patient their immunopathology varies. Treatment of AID in CVID remains a challenge, but based on a better definition of the immunopathology first attempts of targeted treatment have been made. Summary The increasing comprehension of immunological concepts promoting AID in CVID will allow better and in some cases

Journal

Current Opinion in Allergy & Clinical ImmunologyWolters Kluwer Health

Published: Dec 1, 2015

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