Relapsing polychondritis

• What is Relapsing Polychondritis
• Statistics on Relapsing Polychondritis
• Risk Factors for Relapsing Polychondritis
• Progression of Relapsing Polychondritis
• Symptoms of Relapsing Polychondritis
• Clinical Examination of Relapsing Polychondritis
• How is Relapsing Polychondritis Diagnosed?
• Prognosis of Relapsing Polychondritis
• How is Relapsing Polychondritis Treated?
• Relapsing Polychondritis References

What is Relapsing Polychondritis

Relapsing polychondritis is an uncommon, inflammatory disorder of cartilage which mainly affects the cartilage in the ear, nose and trachea (windpipe). This can result in pain, swelling and eventual destruction of cartilage in the affected structures.

Statistics on Relapsing Polychondritis

Relapsing polychondritis (RP) is thought to be a rare disease, with only 600 or so cases reported worldwide up to 1997.

Risk Factors for Relapsing Polychondritis

RP is thought to be caused by the reaction of the body’s immune system, or defence mechanisms (which normally fight infections) to itself, resulting in damage to cartilage structures in the body. RP may occur in any ethnic group, and in both men and women. It may occur at any age, but onset is typically during middle-age.

Progression of Relapsing Polychondritis

Relapsing polychondritis is characterised by flares of disease that improve over weeks to months. Patients may have repeated episodes of the disease over several years. Eventually the supporting cartilage becomes damaged, resulting in floppy ears, a sloping abnormally shaped nose, and vision, hearing, and balance problems.

How is Relapsing Polychondritis Diagnosed?

Relapsing polychondritis is diagnosed based on clincal and physical findings. Sometimes a biopsy (a sample) of cartilage is needed to confirm RP, but this is avoided where possible.
Other tests which may be helpful include:

• blood tests
• urine analysis
• blood and sputum cultures (if infection is suspected)
• ECG if heart involvement is suspected
• chest x-ray,
• CT or MRI scans if lung involvement is suspected.

 

Prognosis of Relapsing Polychondritis

After 5 years, the survival rate for relapsing polychondritis is thought to lie somewhere between 75-90% depending on the severity of the condition. Death usually occurs due to breathing and airway problems, infections and as a result of damage to blood vessels.

How is Relapsing Polychondritis Treated?

Mild cases of RP are usually treated with with aspirin or anti-inflammatory medications. More severe cases are treated with steroids or an anti-cancer drug called methotrexate. Sometimes suppression of the the immune system with medications is effective.

Relapsing Polychondritis References

1. Harp, Raugi. Relapsing Polychondritis. eMedicine 2004.
   [available online @ http://www.emedicine.com/derm/topic375.htm]
2. Kumar and Clark. Clinical Medicine, 5th ed. WB Saunders, Toronto (2002).
3. The Merck Manual of Diagnosis and Therapy: Relapsing Polychondritis.
   [available online @ http://www.merck.com/mrkshared/mmanual/]
4. Welsh, Meffert. Photo Quiz: Red Ear. Am F Phys.
   [available online @ http://www.aafp.org/afp/20020701/photo.html]