Relapsing Polychondritis

Peter D. Kent; Clement J. Michet, Jr; Harvinder S. Luthra

Disclosures

Curr Opin Rheumatol. 2004;16(1) 

In This Article

Management

Because of its rarity, treatment for relapsing polychondritis is based more on case series than on quality clinical trials. Traditional therapy is with oral corticosteroids, in doses of 10 to 20 mg daily for mild to moderate auricular and nasal chondritis or arthritis, whereas doses of 1 mg/kg/day are used for sensorineural hearing loss, vestibular symptoms, ocular involvement, respiratory compromise, and vascular and renal complications. Pulse intravenous steroids (1 g/day for 3 days) and nebulized racemic ephedrine may be helpful for acute airway obstruction, allowing tracheostomy to be performed electively instead of emergently.[60,61]

Nonsteroidal antiinflammatory drugs may be useful for arthralgias and mild arthritis. Colchicine (0.6 mg twice daily) is of reported benefit particularly for auricular chondritis, for which its effects are noted within days.[62,63] Dapsone (50 to 200 mg/day) may also be of use in milder disease, but side effects are common.[64,65]

One recent report describes a child successfully treated with daily oral fetal bovine type II collagen. There was also a dramatic change in the pre- and posttreatment T-cell cytokine profile. Although little can be gleaned from this single report, the favorable side effect profile oral type II collagen makes it deserving of further study.[66**]

Methotrexate and azathioprine have been used with some success as disease-modifying and steroid-sparing agents.[4,67] Treatment with oral (1 to 2 mg/kg/day) or pulse intravenous (0.6 g/m2) cyclophosphamide is used for organ-threatening pulmonary, cardiac, or renal disease.[68,69] Cyclosporine A, in doses of 5 to 15 mg/kg/day has helped in several cases that were refractory to other agents.[70,71]

Several biologic therapies have been tried in relapsing polychondritis. An anti-CD4 chimeric monoclonal antibody has been successful as a salvage therapy.[72] Infliximab has been reported to induce remission in one patient,[73] but another diabetic patient with relapsing polychondritis developed severe septicemia, a parasternal abscess, and died after initiating infliximab therapy.[74] When other treatments fail, autologous stem cell transplantation may induce complete remission in relapsing polychondritis.[75]

Adjuncts to medication therapy may be required. Continuous positive airway pressure can provide symptomatic benefit, particularly at night, in patients with tracheo- and bronchomalacia.[76] Tracheostomy may be required in the setting of respiratory distress and localized subglottic involvement.[77] Metallic stent placement can provide immediate improvement in airflow dynamics, but complications plague their use.[77,78*,79,80] In extreme cases, surgeries to splint open externally or reconstruct the airway have been tried, but results are mediocre at best.[77]

Prophylactic complete replacement of the ascending aorta with a graft that includes the aortic valve should be performed in patients with severe aortic insufficiency to limit the postoperative risk of periprosthetic leak and aneurysm formation.[81] Patients diagnosed with thoracic aneurysms should be screened for abdominal and iliac aneurysms that may be asymptomatic until they rupture.[20] Permanent pacemaker placement is indicated for complete heart block.[23]

All patients undergoing surgery should have preoperative pulmonary function tests and, if abnormal, CT of the chest to screen for airway involvement. A small endotracheal tube placed over a bronchoscope may limit traumatic intubation or respiratory decompensation. Continuous positive airway pressure can be administered if necessary. Whenever the procedure allows, intubation should be avoided, using local or regional anesthesia.[82]

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