Relapsing polychondritis

Relapsing polychondritis (RP) is a rare multi-systemic disease characterised by recurrent inflammation of cartilaginous structures in the body. It can also affect other proteoglycan-rich structures 1.

The condition is extremely rare with an estimated incidence of ~1 in 285,000. There is no recognised gender predilection although pulmonary involvement may be more common in females 4,7. Patients typically present in middle age and there is equal occurrence in men and women 11

Respiratory symptoms are seen in ~20% of patients at presentation and eventually ~60% will develop respiratory tract involvement which is manifested by a combination of symptoms including laryngeal tenderness, hoarseness, dyspnoea, and stridor/wheeze.

An auto-immune mediated mechanism has been postulated.

Distribution 

Commonly affected areas include:

  • tracheobronchial tree: present in up to 50% of cases 2-3
  • external ear cartilages
  • nasal cartilages 
  • peripheral joints
  • laryngeal cartilages 

Other areas that can be involved include 8:

  • uvea: uveal inflammation
  • cardiovascular system: aortitis/arteritis
CT chest
  • increased airway wall attenuation: common 4
  • smooth anterior and lateral tracheal wall thickening with sparing of the posterior membranous wall: if present is considered virtually pathognomonic 2
  • luminal narrowing: tracheobronchial and peripheral bronchial
  • accompanying dense tracheal cartilage calcification
  • sub-glottic stenosis
  • dynamic imaging may demonstrate airway collapse best seen at end expiratory phase (dynamic tracheal collapse)
  • lobar air trapping 
  • bronchiectasis: uncommon 
  • lymphadenopathy is generally not a feature 9 but may be present in some cases 12

Many patients have a fluctuating but progressive course. Most morbidity and mortality is due to respiratory involvement (frequent respiratory infection and airway collapse). Medical management (with corticosteroids, azathioprine, cyclosporin) is the mainstay of treatment. Selected surgical options include 7:

  • tracheostomy: for localised upper airway involvement
  • endobronchial polymeric silicone stent placement to aid/maintain airway patency

The differential will somewhat depend on the spectrum of organ involvement.

For tracheal and bronchial thickening consider:

For tracheal narrowing consider: differential of diffuse tracheal narrowing

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Article Information

rID: 11031
Section: Pathology
Tag: cases
Synonyms or Alternate Spellings:
  • Atrophic polychondritis
  • Relapsing polychondritis (RP)
  • Relapsing polychondritis (RPC)

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