Relapsing polychondritis
C. Prescribe prednisone, 0.25 to 1 mg/kg orally daily
The key to the correct diagnosis is recognizing that auricular inflammation and scleritis are characteristic of relapsing polychondritis. Although chest radiography and PFT (choice A) are recommended when evaluating patients for relapsing polychondritis, these tests should not delay treatment. A biopsy of the left ear (choice B) is unnecessary if the diagnosis of relapsing polychondritis can be made based on clinical presentation. Ibuprofen (choice D) is incorrect because relapsing polychondritis affecting the eyes should be treated with steroids.
Relapsing polychondritis is a rare, progressive, relapsing-remitting autoimmune disease that affects cartilaginous and proteoglycan-rich tissues throughout the body, and it results in recurrent episodes of inflammation that can destroy tissue. The incidence of relapsing polychondritis is approximately 0.7 to 3.5 cases per million per year.1,2 Disease onset typically occurs in middle age, and women appear slightly more affected than men.1,2 Approximately one-third of patients have another systemic illness, such as vasculitis, connective tissue disease, or myelodysplastic syndrome.1,3 Due to the frequent spontaneous resolution of flares and variability of presentation, the diagnosis of relapsing polychondritis is often delayed for years after initial symptom onset.3,4