August 2017 | Case of the Month

The diagnosis is relapsing polychondritis. 

Relapsing polychondritis is a rare, non‐infective condition, characterised by severe, episodic, inflammation of cartilaginous structures, predominantly those of the ears, nose, and respiratory tract, leading to destruction of cartilage and fibroblastic regeneration. Other affected structures include the eyes, cardiovascular system, and the peripheral joints.

The cause is largely unknown although it appears to have an autoimmune basis, as relapsing polychondritis is associated with a number of conditions such as rheumatoid arthritis, lupus erythematosus, vasculitis, Behçet's disease, Hashimoto's disease, and inflammatory bowel disease.

Relapsing polychondritis is most common in whites, and perhaps has a slight female predominance. It usually begins between the ages of 30 and 50 years.

Chondritis ultimately involves three or more sites in most patients, but may be limited to one or two for long periods. The clinical features can include:

• Ears
  • Up to 95% of cases develop auricular chondritis
  • During the acute stage the affected area is swollen, red and tender, and may be mistaken for cellulitis, although sparing of the earlobe is a useful differentiating sign
  • Over time, as the cartilage is destroyed, the ear becomes floppy (the ‘forward listening’ ear)
  • Audiovestibular damage can cause a sudden loss of hearing, which is usually permanent
     
• Nose and respiratory tract
  • Are the second most commonly affected sites
  • Acute inflammation of the nasal cartilage causes pain and a sense of fullness over the nasal bridge, along with obstructive symptoms. Longer-term involvement leads to a saddle‐nose deformity, with collapse of the nasal bridge
  • Inflammation may involve any portion of the respiratory tract. Involvement of the tracheal cartilage results in cough, shortness of breath, hoarseness, and stridor/wheeze
  • Recurrent chest infections are common
  • The upper airways can eventually become stenosed and permanent tracheostomy may be required
     
• Musculoskeletal
  • Up to 75% of cases are affected
  • Most commonly, the arthritis is asymmetric, oligoarticular or polyarticular, and usually non-erosive
  • The ankles, elbow, wrists, proximal interphalangeal joints, metacarpophalangeal joints, and metatarsophalangeal joints are often involved. The costochondral, and sternoclavicular joints may also be involved
     
• Ocular abnormalities are found in more than 50% of cases
  • Ocular abnormalities are found in more than 50% of cases
  • There are a wide range of presentations, with episcleritis and scleritis being the most common. Vision can be compromised in severe episodes
     

Refer to the chapter on relapsing polychondritis for more information.