Relapsing Polychondritis

Relapsing polychondritis is a rare but serious disease of the cartilage and other tissues with similar proteoglycan chemical structure. It often starts with nonspecific symptoms including fever and malaise, similar to the flu.

On the average, it takes about three years after symptoms begin to get the diagnosis. Most patients are in the age range 40 to 60 years. The disease is called relapsing because patients feel better and then worse, perhaps for many years.

Inflammation may cause damage to any of the following areas:

1. External ear. This is the most common initial symptom.
2. Nose. The saddle nose deformity is a sign of serious disease. It is caused by collapse of the dorsum of the nose.
3. Peripheral joints. Seronegative, nonerosive arthritis is one of the diagnostic criteria.
4. Airway, including the larynx and tracheobronchial tree.
5. Eyes. Inflammation of the conjunctiva, cornea, sclera, or choroid may occur.
6. Heart and blood vessels.
7. Inner ear. Lesions may form in the inner ear and labyrinth.

Drugs used to treat relapsing polychondritis include:
1. Prednisone, a synthetic corticosteroid.
2. Mycophenolate mofetil (CellCept), an immunosuppressive drug used for organ transplants.
3. Etanercept (Enbrel), a tumor necrosis factor inhibitor used for inflammatory and autoimmune diseases.
4. Infliximab (Remicade), another tumor necrosis factor inhibitor.
5. Tocilizumab (RoACTEMRA), an anti-interleukin-6 receptor antibody.
6. Methotrexate, an antimetabolite agent that interferes with the growth of certain types of cells.

A recent article in the medical journal Chest reports that airway involvement is one of the worst possibilities for relapsing polychondritis cases. Even with aggressive drug treatment, many patients require surgery. Respiratory symptoms begin with shortness of breath, cough, wheezing, hoarseness, and chest discomfort. These symptoms were found in 21 percent of the patients in the study, and progressed to respiratory failure in some.

Surgical treatments include:
1. Balloon dilatation,
2. Stenting with metallic or silicone stents,
3. Tracheotomy.

Computed tomography (CT) scans and bronchoscopy are recommended diagnostic tools for evaluating damage to the respiratory system. A specialized rheumatology center, such as the one at Mayo clinic, is recommended for treatment.

Sources:

Pol K et al, “Relapsing polychondritis: case report and literature review”, Pol Arch Med Wewn. 2009 Oct; 119(10): 680-3.

Ernst A et al, “Relapsing polychondritis and airway involvement”, Chest. 2009 Apr; 135(4): 1024-30.

More details online:
http://emedicine.medscape.com/article/331475-overview

Mayo Clinic offers treatment:
http://www.mayoclinic.org/rheumatology/

Linda Fugate is a scientist and writer in Austin, Texas. She has a Ph.D. in Physics and an M.S. in Macromolecular Science and Engineering. Her background includes academic and industrial research in materials science. She currently writes song lyrics and health articles.

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