musculoskeletal and connective tissue disorders
Relapsing Polychondritis
Treatment of Relapsing Polychondritis
Nonsteroidal anti-inflammatory drugs ordapsonefor mild ear disease Corticosteroids Sometimesmethotrexateor other immunosuppressants (eg,cyclosporine,cyclophosphamide,azathioprine, anti-tumor necrosis factor drugs) Mild recurrent ear disease may respond to nonsteroidal anti-inflammatory drugs in anti-inflammatory doses, or oraldapsone(50 to 100 mg once a day). However, most patients are treated with oralprednisone30 to 60 mg once a day, with tapering of the dose as soon as there is a clinical response. Some patients require chronic use. In such patients, oralmethotrexate15 to 20 mg once a week can reduce the requirement for corticosteroids. Very severe cases may require other immunosuppressants, such ascyclosporine,cyclophosphamide, anti-tumor necrosis factor drugs (eg,infliximab,etanercept), orazathioprine. None of these therapies has been tested in controlled trials or has been shown to decrease mortality. If tracheal narrowing causes stridor, a tracheostomy or stent may be needed. (see Prevention ), and vaccines against common infections (eg, Endotracheal intubation can be technically difficult because of tracheal involvement and narrowing; also, intratracheal manipulation can lead to life-threatening postanesthetic deterioration by causing further glottic or subglottic inflammation. Thus, endotracheal intubation should be avoided whenever possible (eg, instead using local and regional anesthesia). When endotracheal intubation is unavoidable, preparations should be made for emergency cricothyrotomy. More extensive tracheobronchial collapse may require tracheal reconstruction. Eye disease may sometimes be recalcitrant to treatment, especially when involving the sclera, and has a poor prognosis. Patients on long-term corticosteroid therapy should receive Prevention . 