ABSTRACT
Relapsing polychondritis (RP) is a rare disease causing inflammation and destruction
of cartilage and other connective tissues. Specific laboratory aberrations are lacking.
Predominant clinical manifestations include auricular chondritis, polyarthritis, nasal
chondritis, ocular inflammation, audiovestibular damage, and respiratory tract chondritis.
A relapsing course is characteristic. Airways are involved in 50% of patients and
may cause dyspnea, stridor, wheezing, hoarseness, aphonia, and laryneal or tracheal
tenderness. Airflow obstruction may result from RP involving the tracheobronchial
tree; there is no interstitial or pulmonary vascular component. Collapse or failure
of the trachea to dilate during inspiration is a key feature. Fast computed tomographic
(CT) scanners can visualize dynamic airway collapse. Randomized, controlled trials
of therapy have not been done. Corticosteroids and nonsteroidal anti-inflammatory
drugs (NSAIDs) are used most commonly, but optimal regimens and duration of therapy
have not been elucidated. Endobronchial stents or tracheostomy may be required for
severe stenoses refractory to medical therapy.
KEYWORD
Relapsing polychondritis - chondritis - cartilage - tracheal stenosis - airway collapse
