Vasculitis and Relapsing Polychondritis

Relapsing polychondritis (RPC) is an autoimmune disorder characterized by multisystem involvement of cartilaginous structures. The pathogenesis of this disease is not well understood. Vasculitis in RPC may involve large, medium or small-sized arteries. Aortic disease in RPC may result in aneurysm formation or dissection. Other vasculitic manifestations including scleritis, episcleritis, retinal vasculitis, cutaneous vasculitis, coronary vasculitis and cerebral vasculitis have all been reported in association with RPC. In up to 10% cases, RPC may be associated with systemic vasculitis, mainly granulomatosis with polyangiitis (Wegener's) and microscopic polyangiitis (MPA). Corticosteroids and immunosuppressive medications remain the mainstay of therapy. RPC patients with an associated systemic vasculitis have a poorer prognosis.