What Is Relapsing Polychondritis?

Relapsing polychondritis (RP) is a systemic inflammatory disease of unknown etiology that can be fatal. The disease affects multiple organs, particularly cartilaginous structures such as the ears, nose, airways and joints as well as eyes, skin, heart valves and brain.

DIAGNOSIS

  • Relapsing polychondritis (RP) is extremely difficult to diagnose.
  • There is no available blood test to make the diagnosis.
  • The diagnosis is based on clinical symptoms and signs that fit the pattern consistent with RP.
  • If RP is not diagnosed early and treated effectively, there can be irreversible damage and long term complications.

Symptoms of relapsing polychondritis are variable and may include:

  • General malaise, low grade fever, loss of appetite
  • Redness and pain on the eyes
  • Inflammation, redness, swelling and/or pain in outer (cartilaginous) portions of the ear
  • Sore throat, anterior neck pain, hoarseness
  • Shortness of breath, wheezing, dry cough
  • Hearing loss, tinnitus, dizziness
  • Pain and redness on the nose
  • Skin lesions
  • Joint pain and swelling
  • Rib pain, sternum pain

Possible Complications:

  • Disability
  • Blindness
  • Tracheomalacia and bronchomalacia
  • Subglottic stenosis
  • Ear and nose deformities
  • Hearing loss

TREATMENT

The primary goals of treatment for people with RP are to relieve present symptoms and to preserve the structure of the affected cartilage.  Relapsing polychondritis is a complex condition that requires a team approach for patient care, as follows:

Dermatologists or specialists in infectious diseases are often involved early in the course of the disease to evaluate the patient for infectious causes of cellulitis or perichondritis.

Rheumatologists usually become the primary care provider and should be involved early in patient care.

Ophthalmologists should also be involved early to diagnose, monitor, and treat the potentially devastating ocular complications.

Cardiologists, neurologists, nephrologists, and otolaryngologists may be asked to manage other aspects of relapsing polychondritis.

Plastic surgeons can aid in nasal reconstruction if saddle-nose deformity is present.

Information For Physicians and Other Health Professionals

  • RP should be suspected in a patient with sign and symptoms suggesting inflammation of three of the six following: ears, nose, tracheobronchial tree, eyes, inner ear and joints.
  • Early recognition of the disease can improve morbility and mortality.
  • Patients with RP require a multidisciplinary medical team in order to provide the best possible care.

Frequently Asked Questions

It was initially thought that all patients with RP would have severe health problems due to the disease. It is now known that there is a great deal of variation in the severity of the disease from person to person. Many individuals may have mild symptoms, which recur infrequently, while others may have persistent problems that become debilitating or life-threatening. At this time it is not known how to predict how the disease will affect any specific individual.

Treatment for relapsing polychondritis is approached in a manner similar to other autoimmune diseases. A team approach is very helpful in treating this disease as it can involve many organs. Initial treatment usually includes prednisone and further medical treatment should be done with the supervision of a rheumatologist and may include other medications. Treatment of eye disease should be done in consultation with an ophthalmologist, and those individuals with respiratory symptoms may need to be followed by an otolaryngologist or pulmonologist.

Although RP is rare, medical research is being conducted in the US and Japan to try to expand understanding of the disease. The current data about the mechanism of the disease, as well as the best treatment approach, is very limited. In order to improve patient outcomes with RP, research is necessary to gain a better understanding of the disease, develop a classification of disease severity, and to develop better treatment options.

RP is an uncommon disease that affects approximately 3-5 individuals per million people. RP affects both men and women. It can affect individuals of all ages – it has been described in childhood and beginning as late as age 80, but it most frequently occurs midlife.

You should talk to your doctor about being referred to a rheumatologist, a specialist in treating conditions such as RP, rheumatoid arthritis, and lupus. The rheumatologist may need to perform different tests to rule out other conditions.