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Refractory relapsing polychondritis: challenges and solutions

Relapsing polychondritis is a severe systemic immune-mediated disease characterized by an episodic and progressive inflammatory condition with progressive destruction of cartilaginous structures. This disease has for nearly a century kept secrets not yet explained. The real incidence and prevalence...

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Autores principales: Lekpa, Fernando Kemta, Chevalier, Xavier
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Dove Medical Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5768290/
https://www.ncbi.nlm.nih.gov/pubmed/29391837
http://dx.doi.org/10.2147/OARRR.S142892
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author Lekpa, Fernando Kemta
Chevalier, Xavier
author_facet Lekpa, Fernando Kemta
Chevalier, Xavier
author_sort Lekpa, Fernando Kemta
collection PubMed
description Relapsing polychondritis is a severe systemic immune-mediated disease characterized by an episodic and progressive inflammatory condition with progressive destruction of cartilaginous structures. This disease has for nearly a century kept secrets not yet explained. The real incidence and prevalence of this rare disease are unknown. The multiple clinical presentations and episodic nature of relapsing polychondritis cause a significant diagnosis delay. No guidelines for the management of patients with relapsing polychondritis have been validated to date. The challenges remain, both in the understanding of its pathophysiology and diagnosis, evaluation of its activity and prognosis, and its treatment. Possible solutions involve the sharing of data for relapsing polychondritis from worldwide reference centers. Thus, we would be able to evolve toward a better knowledge of its pathophysiology, the publication of new diagnosis criteria, which will include biological markers and imaging findings, the prediction of life-threatening or organ-threatening situations, and the publication of therapeutic evidence-based guidelines after performing at randomized controlled trials.
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spelling pubmed-57682902018-02-01 Refractory relapsing polychondritis: challenges and solutions Lekpa, Fernando Kemta Chevalier, Xavier Open Access Rheumatol Review Relapsing polychondritis is a severe systemic immune-mediated disease characterized by an episodic and progressive inflammatory condition with progressive destruction of cartilaginous structures. This disease has for nearly a century kept secrets not yet explained. The real incidence and prevalence of this rare disease are unknown. The multiple clinical presentations and episodic nature of relapsing polychondritis cause a significant diagnosis delay. No guidelines for the management of patients with relapsing polychondritis have been validated to date. The challenges remain, both in the understanding of its pathophysiology and diagnosis, evaluation of its activity and prognosis, and its treatment. Possible solutions involve the sharing of data for relapsing polychondritis from worldwide reference centers. Thus, we would be able to evolve toward a better knowledge of its pathophysiology, the publication of new diagnosis criteria, which will include biological markers and imaging findings, the prediction of life-threatening or organ-threatening situations, and the publication of therapeutic evidence-based guidelines after performing at randomized controlled trials. Dove Medical Press 2018-01-09 /pmc/articles/PMC5768290/ /pubmed/29391837 http://dx.doi.org/10.2147/OARRR.S142892 Text en © 2018 Kemta Lekpa and Chevalier. This work is published and licensed by Dove Medical Press Limited The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License (http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed
spellingShingle Review
Lekpa, Fernando Kemta
Chevalier, Xavier
Refractory relapsing polychondritis: challenges and solutions
title Refractory relapsing polychondritis: challenges and solutions
title_full Refractory relapsing polychondritis: challenges and solutions
title_fullStr Refractory relapsing polychondritis: challenges and solutions
title_full_unstemmed Refractory relapsing polychondritis: challenges and solutions
title_short Refractory relapsing polychondritis: challenges and solutions
title_sort refractory relapsing polychondritis: challenges and solutions
topic Review
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5768290/
https://www.ncbi.nlm.nih.gov/pubmed/29391837
http://dx.doi.org/10.2147/OARRR.S142892
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