Refractory relapsing polychondritis: challenges and solutions
نویسندگان
چکیده
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.
منابع مشابه
Successful treatment of relapsing polychondritis with infliximab.
Relapsing polychondritis (RP) is a rare and potentially fatal autoimmune disease in which an inappropriate immune response destroys the cartilage of the ears, larynx and nose. Many therapeutic approaches have been reported. We describe the results obtained with infliximab in a patient with RP unresponsive to conventional therapy. This therapy could be a new weapon to treat refractory RP.
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