How is Relapsing Polychondritis treated
What medications are used in the treatment of Relapsing Polychondritis?
Nonsteroidal antiinflammatory drugs, dapsone, and low-dose prednisone may be used to control minor inflammatory episodes. However, with more active disease, prednisone doses of 20 to 60 mg/day may be required until control is attained. Methylprednisolone pulses (1 g/day × 3 days) are used for severe disease manifestations such as acute respiratory compromise, neurosensory hearing loss, central nervous system involvement, necrotizing scleritis, and systemic vasculitis.
Continued inflammation or an inability to taper glucocorticoids to safe maintenance doses warrants the addition of a steroid-sparing agent. Dapsone (50–200 mg/day) has been useful in patients without major organ involvement. In patients with ocular, pulmonary, or cardiovascular involvement, or with systemic vasculitis, other immunosuppressives such as cyclophosphamide, methotrexate, azathioprine, leflunomide, mycophenolate mofetil, and cyclosporine (when cytopenias present) are used. A recommended approach in patients with severe disease is to control manifestations with corticosteroids and cyclophosphamide and later switch to a less toxic medication like methotrexate. Patients who fail to respond may be candidates for biologic therapies, including infliximab (large number of case reports/series), adalimumab, etanercept, tocilizumab, anakinra, and abatacept. Results with rituximab in RPC have been largely disappointing. Plasmapharesis, intravenous immunoglobulin (2 g/kg per month), and stem cell transplantation have been used as salvage therapies.
