Relapsing Polychondritis

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.

A Retrospective Study of the Relationship Between Tracheal Collapse and Bronchiectasis in Dogs

“…Acquired tracheomalacia is commonly associated with excessive dynamic airway collapse and chronic obstructive pulmonary disease 12,16 . Rarely, acquired tracheomalacia is secondary to relapsing polychondritis, characterized by inflammation and destruction of cartilage and other connective tissue 13,20,21 . Bronchiectasis, irreversible dilation of diseased bronchi, is reported in congenital and acquired forms of tracheomalacia in humans 16–19,21 …”

Respiratory insufficiency and dynamic hyperinflation after rigid bronchoscopy in a patient with relapsing polychondritis -a case report-

“…Diffuse airway involvement precluded effective stenting and only introduced complications. Major complications with stent placement are migration, pneumothorax, persistent cough, retained secretions, infection, hemorrhage, and formation of granulation tissues [9]. Most important thing in stent placement is choosing the location at which the stents will do the most good [10].…”

“…Dyspnea could be aggravated when supine, presumably due to airway collapse [9]. Therefore, this patient tried to encourage breathing with sitting position, but not effective in this case.…”

“…Therefore, we presumed that the near-tampon status was provoked from dynamic hyperinflation (DHI) by expiratory air trapping due to high inspiratory pressure, luminal collapse, rapid RR, and decreased I : E ratio [12]. Primary concern in central airway obstruction is not the limitation of inspiratory air flow, but rather DHI with hypercarbia and hemodynamic collapse [9]. CT of the upper airway can be normal in patients with malacia component only.…”

Otolaryngological aspects of orofacial pain

“…Relapsing polychondritis (RP) is an episodic inflammatory condition of the cartilaginous and non-cartilaginous tissues that causes progressive destruction of the head and neck cartilages, predominantly those of the ear, nose and laryngotracheobronchial tree (Gergely et al 2004). Other affected structures may include the eye, the cardiovascular system, small and large peripheral joints and the middle and inner ear (Lucente et al 1995;Staats et al 2002;Gergely et al 2004). The aetiology is unknown; however, the pathogenesis is most likely autoimmune (Gergely et al 2004).…”