Head and Neck Surgery

RELAPSING POLYCHONDRITIS

Posted by headnecksurgery on April 5, 2009

Relapsing polychondritis is characterized by episodic recurring inflammation of cartilaginous structures that are eventually replaced by granulation tissue and fibrosis. Women (3:1) are more commonly affected than men, and the average age at onset is 47. There appears to be a racial predilection for whites. Defined features of the disease include recurrent chondritis of the auricles, nonerosive inflammatory polyarthritis, chondritis of the nasal cartilages, ocular inflammation, chondritis of laryngeal and/or tracheal cartilages, and cochlear or vestibular damage. Diagnosis requires three of these features without histologic confirmation, two of these features with response to steroids or dapsone, or any one feature with histologic confirmation.

Auricular chondritis and nonerosive arthritis are the most common presenting symptoms of relapsing polychondritis. Auricular chondritis is characterized by the sudden onset of erythema and pain, sparing the lobule, which lacks cartilage. It is the feature presentation in 33% of patients and will develop in 90% of those with the disease. Resolution occurs in 5 to 10 days with or without treatment. Patients may develop conductive hearing loss secondary to collapse of the external auditory canal, and 40% suffer cochlear or vestibular dysfunction, possibly due to vasculitis of the internal auditory artery.

Chondritis of the nasal cartilages develops in 75% of patients and often does not coincide with the auricular involvement. The nasal cartilage chondritis also has a sudden onset and a resolution, which resolves in several days with or without treatment. After the cartilaginous inflammation subsides, deformities result from the loss of cartilage. These are disfiguring in the ear, and often cause a classic saddle deformity of the nose.

Laryngeal involvement presents early with a nonproductive cough, which progresses to hoarseness and stridor. Fifty-three percent of patients with relapsing polychondritis will have respiratory tract involvement during the course of their disease. Diagnostic endoscopy is dangerous in these patients due to the risk of tracheal collapse. With extensive airway involvement, management is difficult even with tracheotomy. In most cases, corticosteroids are the main form of treatment. The antileprosy sulfone dapsone also has helped in some cases, and methotrexate is playing an increased role.

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