Dyspnoea. Airway obstruction on respiratory function tests.
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Contrast enhanced CT thorax demonstrates long segment narrowing of the thoracic trachea with smooth tracheobroncheal thickening and sparing of the posterior tracheobroncheal membrane (white arrows).
There is also increased attenuation of the tracheal and central bronchial cartilages.
Relapsing polychondritis is an autoimmune disease with anti-cartilage autoantibodies production leading to recurrent inflammation of cartilaginous sites including nasal cartilage, auricular cartilage, peripheral polyarthritis, ocular inflammation and tracheobronchial involvement. Chronic airway cartilage inflammation can lead to eventual fibrosis and tracheomalacia which is a major cause of morbidity and mortality.