Relapsing Polychondritis

Relapsing polychondritis is an uncommon, chronic inflammatory disorder of the cartilage. It is characterized by recurrent episodes of painful cartilage inflammation. Affected cartilage includes that of the ears, nose, throat and joints. The symptom course if often unpredictable and varies greatly between patients. Rarely, in more severe cases the eyes, heart and blood vessels may be involved.

 

Causes

The cause of relapsing polychondritis is unknown. It is suspected that environmental, genetic and possibly infectious factors cause an abnormal response by the immune system leading to inflammation directed at cartilage.

 

Symptoms

Typically, symptoms include sudden pain, swelling, redness and tenderness in the affected cartilage site. Common sites include the nose, throat, joints and/or eyes. Fever, fatigue, and weight loss often develop.

Chronic inflammation of the ears and nose can cause deformity (saddle nose deformity and floppy ears) from weakened cartilage. Impaired hearing, balance, and nausea can be caused by inner ear inflammation.

Inflammation of the windpipe, or trachea, can lead to throat pain, hoarseness, and breathing difficulty. In severe causes this may be life-threatening and require assisted breathing methods.

Joint inflammation (arthritis) can cause pain, swelling, and stiffness of the joints, including of the hands, knees, ankles, wrists, and feet.

Eye inflammation can be mild or severe and can cause permanent vision loss.

Other tissues that can develop inflammation include the aorta (which can lead to aneurysm or aortic valve weakness), tissues in or around the heart (myocarditis and pericarditis), the skin (vasculitis), and the nerves from the brain (cranial nerve palsies).

 

Diagnosis

A thorough medical history and physical examination is important in diagnosing relapsing polychondritis. Cartilage inflammation is often times confused for infection causing individuals to have received multiple course of antibiotics prior to diagnosis. Recognition of the classic pattern of cartilage involvement will lead the physician to the correct diagnosis.

There is no one specific test for diagnosing relapsing polychondritis. Blood tests that indicate inflammation, such as an elevated erythrocyte sedimentation rate (ESR), C-reactive protein, and others, are characteristically abnormal.

If tissue cartilage is biopsied, the involved cartilage will demonstrate non-specific signs of inflammation.

 

Treatment

For mild disease, nonsteroidal anti-inflammatory medications (NSAIDs) including ibuprofen (Motrin), naproxen (Naprosyn), among others, can be helpful to suppress inflammation.

Typically, however, corticosteroids medications (steroids such as prednisone and prednisolone) are required. Initially high-dose corticosteroids are necessary, especially for severe manifestations such as eye or breathing airway involvement.

Methotrexate (Rheumatrex, Trexall) and azathioprine (Imuran) in combination with steroids are well accepted treatments for relapsing polychondritis.

More recently there are reports of use of biological agents called TNF inhibitors (such as infliximab, etanercept, and adalimumab OR Remicade, Enbrel, and Humira, respectively) for more severe cases.

 

Prognosis

The course and symptoms of relapsing polychondritis are often unpredictable.

Repeated bouts of cartilage inflammation frequently leads to permanent destruction of the involved tissues and results in disability. Destruction of nose and ear cartilage results in deformity, and can impair breathing when the trachea is affected.

For most patients the disease is mild. However, rarely relapsing polychondritis is potentially life- threatening. Inflammation of the cartilage of the windpipe (trachea), heart, aorta and other blood vessels can be fatal. Close monitoring of symptoms with a qualified doctor is recommended for optimal results.