Refractory relapsing polychondritis: challenges and solutions

Abstract

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.

Keywords: biologics; criteria; diagnosis; disease activity; relapsing polychondritis; treatments.

Figure 1

Relapsing polychondritis: auricular chondritis during acute phase (A); chronic phase with collapse of the cartilage of the upper pole of the ear (B); saddle nose deformity (C); episcleritis (D). Note: Reproduced with permission from Club Rhumatismes et Inflammations. [website on the Internet]. Available from http://www.cri-net.com/autres-projets/base_images/display_rub.asp?rub=poly_atro.