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Relapsing polychondritis

Relapsing polychondritis (RP) is an uncommon and severe episodic inflammatory condition involving cartilaginous structures, predominantly those of the ear, nose, and laryngotracheobronchial tree. Other affected structures may include the eye, cardiovascular system, peripheral joints, middle ear, and inner ear. In 1923, Jaksch-Wartenhorst described a patient who experienced an 18-month course of progressive degeneration of the peripheral joints, external ears, nasal septum, external auditory canals, inner ear, and epiglottis. He termed this condition polychondropathia.

In 1960, Pearson, Kline, and Newcomer reviewed 12 cases and expanded the clinical spectrum of RP to include nonconcurrent inflammation of the auricles, nasal septum, peripheral joints, and larynx, with occasional involvement of the middle and inner ears, the eyes, costal cartilages, spine, trachea, bronchi, and epiglottis. They noted that after a few episodes of inflammation, the cartilage was replaced by fibrous connective tissue. The term relapsing polychondritis was introduced in that review.

Symptoms of Relapsing polychondritis

Typically, relapsing polychondritis causes sudden pain in the inflamed tissue at the onset of the disease. Common symptoms are pain, redness, swelling, and tenderness in one or both ears, the nose, throat, joints and/or eyes. Fever, fatigue, and weight loss often develop.

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. This is a potentially dangerous area of inflammation in patients with relapsing polychondritis which can require assisted breathing methods when severe.

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 damage vision. Cataracts can be caused by the inflammation or from the cortisone used to treat relapsing polychondritis (see below).

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 of Relapsing polychondritis

A characteristic array of symptoms and physical findings will yield a diagnosis of relapsing polychondritis. Laboratory tests are sometime helpful. Biopsies of the affected cartilage may confirm the diagnosis. Further diagnostic test are done to confirm other associated conditions such as rheumatoid arthritis. It is important to evaluate the airway, although only 10% of patients will die from airway complications.

Treatment of Relapsing polychondritis

Mild inflammations can be treated with aspirin or nonsteroidal anti-inflammatory drugs (NSAIDs) such as ibuprofen. Corticosteroids (most often prednisone) are usually prescribed for more advanced conditions and do improve the disease. They may have to be continued over long periods of time, in which case their usage must be closely watched to avoid complications. Immune suppression with cyclophosphamide, azathioprine, cyclosporine, or dapsone is reserved for more aggressive cases. A collapsed chest or airway may require surgical support, and a heart valve or aorta may need repair or replacing.

Prognosis of Relapsing polychondritis

There is no known cure for relapsing polychondritis. It can only be combated with each onset of inflammation and deterioration of cartilaginous tissue. As the disease progresses over a period of years, the mortality rate increases. At five years duration, relapsing polychondritis has a 30% mortality rate


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