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.

This chapter is set out as follow:

• 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:

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

Eyes

• 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

Other associated features

• Cutaneous and systemic vasculitis
• Giant cell myocarditis, involvement of the heart valves may cause serious complications, including sudden valve rupture, even in a patient otherwise in remission
• Mouth and genital ulcers with inflamed cartilage (the MAGIC syndrome) - characterised by an overlap of relapsing polychondritis with Behçet's disease
• Relapsing polychondritis has been seen in patients with underlying myelodysplastic syndrome and, less often, lymphoma. These cases may be paraneoplastic in nature

• Blood tests
• Anaemia is common. Leukocytosis is inconstant, but eosinophilia is found in 40% of cases
• Plasma viscosity or ESR is usually raised
• The rheumatoid factor and antinuclear factor are often positive

• Radiology
  • Radiological abnormalities are not pathognomonic, but evidence of extensive destruction of joint cartilage without changes in adjacent bone is suggestive on plain X‐ray

• Progression of acute relapses can be controlled with systemic corticosteroids

• Other drugs - indomethacin and dapsone have been used and colchicine is also helpful in some patients. Immunosuppressive drugs such as methotrexate and ciclosporin may have a role as steroid sparing therapies

• Surgical reconstruction of the nose or larynx is sometimes required

• Prognosis - the course of the disease is extremely variable. Some cases continue to relapse for over 20 years, but others become inactive within a short period. Pregnancy does not appear to affect the course of the disease, although complications are more frequent. Deformity of the ears and nose is common, but in general the disease is a source of discomfort and disfigurement rather than life-threatening