146 Biologics in the treatment of relapsing polychondritis

Abstract

Background: Relapsing polychondritis is a complex autoimmune disease characterised by inflammation of proteoglycan rich structures and cartilaginous tissues. There have been several case reports describing the use of biologics in relapsing polychondritis. We aim to assess the efficacy and safety of biologics in patients with refractory relapsing polychondritis. Method(s): We retrospectively reviewed five patients with relapsing polychondritis who were treated with biologics. The medical records and laboratory results of all the patients were reviewed. Clinical details recorded include inflammatory markers [Erythrocyte Sedimentation Rate (ESR) and C-reactive protein (CRP)], corticosteroid dosages, Relapsing Polychondritis Disease Activity Index (RPDAI) at baseline and three months. Result(s): All the patients were females and majority were Caucasians (80%). The median age was 48 years old (range 42-54 years old). The median disease duration prior to commencement of the first biologic therapy was four years (range 2-16 years). All patients had nasal and auricular chondritis, four patients had laryngotracheal involvement, four patients had musculoskeletal involvement, two patients had ocular involvement (scleritis and episcleritis) and one patient had cutaneous involvement (lobular panniculitis). Three patients were treated with anti-tumour necrosis factor alpha (anti- TNFa; 2 infliximab and one adalimumab) and two patients with rituximab as the first line biologic agent. One patient was treated with anti-TNFa as the second line biologic agent following rituximab. Treatment received prior to the first biologic agent were corticosteroids and intravenous cyclophosphamide (Euro-Lupus regime) in all the patients, methotrexate (four patients), mycophenolate mofetil (two patients), azathioprine (two patients), hydroxychloroquine (two patients) and cyclosporine (one patient). The pre-biologic (median) prednisone dose, CRP, ESR and RPDAI were 30 mg, 9.5 mg/L, 31.5 mm/hr and 26.5 respectively. The main indications for initiation of biologic therapy were refractory laryngotracheal disease (two patients), inflammatory arthritis (one patient), laryngotracheal disease and inflammatory arthritis (one patient) and refractory vasculitic ulcer (one patient). The post-biologic (median) prednisone dose, CRP, ESR and RPDAI were 15 mg, 5 mg/L, 13 mm/hr and 19.5 respectively. One patient failed both rituximab and anti-TNFa with persistent laryngotracheal disease. One patient experienced loss of efficacy to anti-TNFa therapy at three months. One patient had sustained disease remission at 44 months after initiation of anti-TNFa therapy. There was one death recorded. Conclusion(s): Biologic therapy appears to be promising in the treatment of relapsing polychondritis that is resistant to conventional immunosuppresants.