Relapsing Polychondritis and Large-vessel Vasculitis

Relapsing polychondritis (RP) is a rare and in some cases fatal systemic inflammatory rheumatic disorder characterized by episodic inflammation of cartilage1,2,3. Common clinical features include chondritis of the nasal bridge, auricular cartilage, ocular and inner ear inflammation, arthritis, and involvement of the tracheobronchial tree. Destruction of the laryngeal and tracheal cartilage rings may lead to collapse of the airways and is associated with a high risk of morbidity and mortality4. Its rarity often leads to considerable delay in establishing a diagnosis1,2,3. RP may present with similar clinical features to other autoimmune rheumatic diseases such as granulomatosis with polyangiitis (GPA) and eosinophilic GPA (eGPA). Treatment for RP is usually with corticosteroids and immunosuppressive drugs but there are no randomized trials or specific guidelines for management, so treatment remains empirical and based on expert opinion5. Vascular involvement in RP ranges from 5% to 25%. The disease can affect small, medium, and large vessels. Although aortic involvement is particularly rare, it is associated with significant morbidity and mortality. The largest study in the literature evaluating aortic involvement by Le Besnerais, et al of 172 patients with RP found a prevalence of nonatheromatous aortic disease in 11 patients (6.4%)6. The pattern of … Address correspondence to Prof. D. D'Cruz, Consultant Rheumatologist, Louise Coote Lupus Unit, Guy's Hospital, London SE1 9RT, UK. Email: david.d'cruz{at}kcl.ac.uk.