Relapsing Polychondritis

Relapsing polychondritis is discussed in detail in Chapter 7. It is a chronic multisystem inflammation of cartilage (9). It may affect all types of cartilage including elastic cartilage of ears and nose, hyaline cartilage of joints, and tracheobronchial cartilage. About one-half of patients have airway involvement. The female-to-male ratio is 3:1. Peak onset is in the fourth to fifth decades. It occurs predominantly in Caucasians but occasionally in other races. Relapsing polychondritis can also affect other tissues such as the eyes, blood vessels, heart, and inner ear. It most often presents with sudden onset of auricular pain and erythema that spare the lobule, accompanied by fever and lethargy. Acute episodes resolve within 5 to 10 days, but recurrent bouts of inflammation are common, with progressive permanent destruction of the cartilage (Fig. 4).

Diagnosis is based primarily on the history and physical because biopsy usually shows nonspecific necrosis. A biopsy of perichondrium at the edge of the lesion may occasionally document the early stage of inflammation. Biopsy is indicated to exclude other conditions such as Wegener's granulomatosis. Conclusive diagnosis requires three of six features: bilateral auricular chondritis, nonerosive seronegative arthritis, nasal chondritis, ocular inflammation, respiratory tract chondritis, and audiovestibular damage.

Symptoms of laryngeal involvement include hoarseness, throat pain, and pain on phonation. Progressive loss of laryngeal cartilage impairs the airway and may cause emergent airway obstruction (Fig. 4).

Steroids provide dramatic response, but side effects limit long-term use. Other drugs that can be effective include dapsone, azathioprine, cyclophosphamide, cyclosporine, and penicillamine. Plasma exchange is also used. Surgical reconstruction of the airway is not effective, and the efficacy of tracheotomy is limited due to diffuse collapse of the trachea. Airway disease can progress to death from pneumonia or obstructive respiratory failure.

FIGURE 4 Resorption of laryngeal and tracheal cartilage, with granulation tissue over tracheotomy tract.

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