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Relapsing Polychondritis

Background

Relapsing polychondritis (RP) is a rare episodic or progressive condition caused by inflammation of cartilage or other connective tissue, such as the ears, nose, throat, joints, kidney and heart. The damage caused by RP may lead to impaired use of these organs. RP occurs equally in middle-aged males and females and mainly occurs in white individuals.

Credits

Medical text written October 2002 by Contact a Family. Approved October 2002 by Dr G Hughes. Last updated March 2006 by Professor G Hughes, Consultant Rheumatologist, London Lupus Centre, London Bridge Hospital, London, UK.

What are the symptoms?

The initial symptoms of RP may begin suddenly. The most common first symptom is pain and swelling of the ear. Both ears may turn red or purple and are tender to the touch. Swelling may extend into the ear canal causing loss of hearing, ear infections, balance disturbances with vertigo and vomiting, and eventually a droopy ear. Throat pain may occur leading to hoarseness and difficulty talking. The nose may be affected and deterioration may lead to a flattened nose bridge (sometimes known as a 'saddle nose'). Inflammation of the eye occurs causing impaired vision. Fatigue and weight loss are common symptoms in RP and fever frequently accompanies acute flares.

During the later stages, the symptoms of RP may become more debilitating and life-threatening. RP may cause deterioration of the cartilage holding the windpipe open and this may lead to difficulties breathing. Deterioration of the rib cartilage can lead to the collapse of the chest, again hindering breathing. Joints everywhere are involved in episodes of arthritis (see entry, Arthritis (Juvenile Idiopathic)), with pain and swelling. As the disease progresses over a period of years, the mortality rate increases. Kidney failure (see entry, Kidney disease) may lead to death.

The disease may occur episodically with complete remission between episodes, or it may continue over time, causing progressive destruction (atrophy) of organs. Individuals may have persistent symptoms between acute flares or the pattern of disease may be more limited. The severity and the frequency of symptoms associated with RP varies between people and with time. Furthermore, the areas affected by RP may either remain constant or be completely unpredictable for each person.

What are the causes?

Cartilage is a tough, flexible tissue that changes into bone in many places in the body. Before birth, all bones start out as cartilage. Although children have more cartilage than adults, cartilage persists in adults in the linings of joints, the nose, the ears, the airway and the ribs near the breast bone. All these areas of the body may be affected by RP.

For many individuals, the cause of their condition remains unknown. However, RP is thought to be related to an underlying immune problem.

How is it diagnosed?

Individuals may have a wide array of symptoms that often pose major diagnostic dilemmas and RP may be misdiagnosed or under diagnosed. RP is frequently diagnosed along with rheumatoid arthritis, systemic lupus erythematosus (see entry Lupus), and other connective tissue diseases.

How is it treated?

Although there is no cure for RP, in many patients the disease is well controlled with methotrexate, with acute flares being controlled with steroids.

Inheritance patterns and prenatal diagnosis

Is there support?

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