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Semin Respir Crit Care Med 2002; 23(2): 145-154
DOI: 10.1055/s-2002-25303
Copyright © 2002 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel.: +1(212) 584-4662

Relapsing Polychondritis

Bruce A. Staats1 , James P. Utz1 , Clement J. Michet Jr.2
  • 1Mayo Clinic and Foundation, Divisions of Pulmonary and Critical Care, Rochester, Minnesota, and Mayo Clinic and Foundation, Division of Rheumatology, Rochester, Minnesota
Further Information

Publication History

Publication Date:
24 April 2002 (online)

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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

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