Chest ImagingRelapsing polychondritis
rare disease of unknown aetiology, characterized by recurrent inflammatory episodes that affect cartilages of the ears, nose, larynx, trachea and joints. The result is fragmentation of the cartilages which are replaced by
fibrosis. Auricular chondritis is the most common manifestation. The respiratory tract is affected in approximately half of patients. Involvement of the airways is potentially an immediately life-threatening condition due to airway
obstruction and carries a long-term poor prognosis. At the early stage of the disease, mucosal oedema is present, resulting in narrowing of the airways. When the cartilages dissolve, the airways become more collapsible and when cartilages are destroyed they are replaced by fibrotic tissue, and fixed
stenosis occurs. Airway
obstruction may induce
atelectasis and recurrent
pulmonary infections. Radiologically, the appearance is that of a
diffuse thickening of the tracheobronchial wall with
diffuse smooth narrowing of the trachea and main bronchi. In addition,
CT may demonstrate stenoses and wall thickening involving the lobar, segmental and subsegmental bronchi. Occasionally
calcification within the tracheobronchial wall is present (
Fig.1) (see
bronchial stenosis).
PG
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CT scan in a patient with relapsing polychondritis. The tracheal walls are thickened and contain calcifications, and the lumen of the trachea is irregular, deformed and narrowed.
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Relapsing polychondritis, Fig.1 | |