Relapsing polychondritis

Last revised by Sebastian Röhrich on 6 Sep 2023

Relapsing polychondritis is a rare multisystem disease characterized 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. 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, dyspnea, and stridor/wheeze.

An autoimmune-mediated mechanism has been postulated.

Commonly affected areas include:

Other areas that can be involved include 8:

  • uvea: uveal inflammation

  • cardiovascular system: aortitis/arteritis

In the 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

  • subglottic 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, NSAIDs, azathioprine, cyclosporine) is the mainstay of treatment.

Selected surgical options include 7:

Tracheobronchial abnormalities that also spare the posterior wall:

Tracheobronchial abnormalities that involve the posterior wall:

  • granulomatosis with polyangiitis (GPA)

    • circumferential involvement

    • the subglottic trachea is the most commonly affected area (involvement down to the main bronchi is possible)

    • ulcerations of the trachea are possible

  • tracheobronchial amyloidosis

    • circumferential involvement

    • may appear as focal or diffuse narrowing

    • calcifications of the tracheal wall can be seen

  • post-intubation tracheal stenosis

    • focal involvement

    • irregular and concentric stenosis

For tracheal narrowing consider: differential of diffuse tracheal narrowing

ADVERTISEMENT: Supporters see fewer/no ads

Cases and figures

  • Case 1
    Drag here to reorder.
  • Case 2
    Drag here to reorder.
  • Case 3
    Drag here to reorder.
  • Case 4
    Drag here to reorder.
  • Case 5
    Drag here to reorder.
  • Updating… Please wait.

     Unable to process the form. Check for errors and try again.

     Thank you for updating your details.