A large-scale survey of patients with relapsing polychondritis in Japan

Abstract

Relapsing polychondritis (RP) is a multisystem disorder characterized by recurrent inflammation and destruction of cartilage. The aim of this study is to assess the clinical characteristics of patients with RP in Japan, which remain unclear. A survey was sent to 395 experienced clinicians who worked in Japanese major institutions. The questionnaire was designed to assess patients' profiles, clinical features, diagnosis, treatments and present complications. The response rate was 30.6% and 239 RP patient data were collected. The average age of onset diagnosis was 52.7 years (range, 3-97) and the male-to-female ratio was 1.1:1. Clinical features of patients with RP in Japan were similar to previous studies. Airway and cardiac involvement, both of which were potentially serious complications of RP, were observed in 119 (49.8%) and 17 patients (7.1 %), respectively. Four patients (1.7%) had myelodysplasia. In addition to oral prednisolone (91.6%), patients received methotrexate (19.7%), cyclophosphamide (12.6%) and cyclosporine (8.4%) with clinical response rates of 64.0%, 66.7% and 73.7%, respectively. 42 patients (17.6%) required and underwent tracheotomy, including 12 patients (5.0%) who were treated with prednisolone only. 22 patients (9.2%) underwent stent placement and/or tracheotomy. The overall mortality rate was 9.0% (22 patients) and respiratory failure and pulmonary infection were the leading causes of death in patients with RP. Airway involvement of RP was fundamentally progressive and required frequent clinical checks and appropriate intervention with administration of both prednisolone and immunosuppressant. Cardiac involvement of RP was less common in Japan as compared with that in Western countries. Rec.11/22/2013, Acc.2/11/2014, pp149-156