What is Relapsing polychondritis
Relapsing polychondritis is a rare disease in which cartilage in many areas of the body becomes inflamed. The disease most commonly affects the ears, nose and the airways of the lungs. The cause is not known, and it occurs most often in people in their 50s or 60s. One theory is that polychondritis might be an autoimmune disease, in which the immune system attacks the body rather than foreign invaders such as viruses. In polychondritis, it’s possible that a triggering event, perhaps an infection, sets off a reaction by the immune system, which unleashes an attack on the body’s cartilage. Some people may have a genetic makeup that makes them more prone to this. The disease does not seem to run in families. It sometimes appears in people who have other disease, such as rheumatoid arthritis, vasculitis (inflammation of blood vessels) and systemic lupus erythematosus (SLE or lupus). Polychondritis is a chronic (long-lasting) disease, although medications frequently can reduce the severity of symptoms. Sometimes, the disease goes into remission, meaning it goes away temporarily, whether or not the person is treated.
Relapsing polychondritis (RP) is characterized by recurrent swelling and inflammation of cartilage and other tissues throughout the body. Cartilage is a tough but flexible tissue that covers the ends of bones at a joint and gives shape and support to other parts of the body. Symptoms of RP include swelling of the cartilage of the ear, nose, and joints. Other parts of the body that may be involved are the airways (trachea), costal (rib) cartilage, eyes, heart, vascular (veins) system, skin, kidney, and nervous system. The signs and symptoms vary from person to person depending on which parts of the body are affected. The exact underlying cause of RP is unknown. There are thought to be genetic and other unknown factors involved. RP often occurs along with autoimmune conditions. Diagnosis is based on the symptoms and clinical examination. Other more common conditions may need to be excluded before RP can be diagnosed. The primary goals of treatment for people with RP are to relieve present symptoms and to preserve the structure of the affected cartilage.
This information is provided by the National Institutes of Health (NIH) Genetic and Rare Diseases Information Center (GARD).
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