The ears or nose may become inflamed and tender.
Other cartilage in the body can be damaged, leading to various symptoms, such as red or painful eyes, hoarseness, cough, difficulty breathing, rashes, and pain around the breastbone.
Blood and laboratory tests, imaging, and removal of a piece of tissue for examination and testing may be done, and established criteria may be used for diagnosis.
If symptoms or complications are moderate or severe, corticosteroids and immunosuppressants usually help.
This disorder affects men and women equally, usually in middle age. The cause of relapsing polychondritis is unknown, but autoimmune reactions to cartilage are suspected.
Symptoms of Relapsing Polychondritis
Typically, one ear or both ears (but not the ear lobes) become red, swollen, and very painful. At the same time or later, a person can develop joint inflammation (arthritis), which may be mild or severe. Cartilage in any joint may be affected, and the cartilage that connects the ribs to the breastbone may become inflamed. Cartilage in the nose is also a common site of inflammation. The nose may become tender, and cartilage can collapse.
Other affected sites include the eyes, resulting in inflammation. Rarely, the cornea may develop a hole (perforation), resulting in blindness. The voice box (larynx), windpipe (trachea), or airways of the lungs can be affected, resulting in hoarseness, a nonproductive cough, shortness of breath, and tenderness over the Adam’s apple. Less often, the heart is involved, leading to heart murmurs and occasionally to heart failure. Rarely, the kidneys are affected.
Flare-ups of inflammation and pain last a few weeks, subside, then recur over a period of several years. Eventually, the supporting cartilage can be damaged, resulting in floppy ears, a sloping saddle nose, and a hollow at the lower part of the chest (pectus excavatum). The nerve in the inner ear can be affected, causing eventual problems with balance and hearing, and eventually vision problems can occur.
People who have this disorder may die if the cartilage in their airways collapses, blocking the flow of air, or if their heart and blood vessels are severely damaged.
Diagnosis of Relapsing Polychondritis
Relapsing polychondritis is diagnosed when a doctor observes at least three of the following symptoms developing over time:
Inflammation of both outer ears
Painful swelling in several joints
Inflammation of the cartilage in the nose
Inflammation of the eye
Cartilage damage in the respiratory tract
Hearing or balance problems
A biopsy of the affected cartilage (most often from the ear) may show characteristic abnormalities but is not required for diagnosis.
Blood tests, such as the erythrocyte sedimentation rate, can detect evidence of inflammation. Blood tests also reveal whether the person has a low number of red blood cells or a high number of white blood cells and whether certain antibodies are present. Although blood test results can help doctors diagnose relapsing polychondritis, they alone cannot confirm a definite diagnosis of relapsing polychondritis because sometimes the abnormalities they detect are present in healthy people or in people who have other disorders.
Doctors also evaluate the airways with spirometry (see Lung volume and flow rate measurements Pulmonary Function Testing (PFT) Pulmonary function tests measure the lungs' capacity to hold air, to move air in and out, and to absorb oxygen. Pulmonary function tests are better at detecting the general type and severity... read more ) and computed tomography Computed Tomography (CT) In computed tomography (CT), which used to be called computed axial tomography (CAT), an x-ray source and x-ray detector rotate around a person. In modern scanners, the x-ray detector usually... read more (CT) of the chest.
Prognosis for Relapsing Polychondritis
Newer therapies have decreased the rate of death, and the survival rate is now 94% after 8 years. People with relapsing polychondritis tend to die earlier than they otherwise would, most often because of damage to the heart, lungs, or blood vessels.
Treatment of Relapsing Polychondritis
Nonsteroidal anti-inflammatory drugs or dapsone for relapsing polychondritis of the ear
Sometimes immunosuppressive drugs
Mild relapsing polychondritis of the ear can be treated with nonsteroidal anti-inflammatory drugs Nonsteroidal Anti-Inflammatory Drugs Pain relievers (analgesics) are the main drugs used to treat pain. Doctors choose a pain reliever based on the type and duration of pain and on the drug's likely benefits and risks. Most pain... read more (NSAIDs) or dapsone. However, most people are given a dose of the corticosteroid prednisone that is gradually decreased as the symptoms begin to lessen. In some people, symptoms do not lessen, so the dose of corticosteroids cannot easily be decreased. These people may also be given methotrexate to reduce the need for corticosteroids.
Sometimes very severe cases are treated with immunosuppressive drugs such as cyclosporine, cyclophosphamide, or azathioprine or drugs that inhibit a chemical called tumor necrosis factor (for example, infliximab or etanercept). These drugs treat the symptoms but have not been shown to alter the ultimate course of the disorder.
Surgery may be needed to correct collapse or narrowing of the trachea.
People who take corticosteroids are at risk of fractures related to osteoporosis Osteoporosis Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more . To prevent osteoporosis, these people are given the drugs used to treat osteoporosis, such as bisphosphonates Drugs Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more and supplemental vitamin D and calcium Calcium and vitamin D Osteoporosis is a condition in which a decrease in the density of bones weakens the bones, making breaks (fractures) likely. Aging, estrogen deficiency, low vitamin D or calcium intake, and... read more .
People who are receiving immunosuppressants are also given drugs to prevent infections such as by the fungus Pneumocystis jirovecii (see prevention of pneumonia in immunocompromised people Prevention Pneumonia is infection of the lungs. Pneumonia in people whose immune system is weakened or impaired (for example, by acquired immunodeficiency syndrome [AIDS], cancer, organ transplantation... read more ) and vaccines against common infections such as pneumonia Pneumococcal Vaccine Pneumococcal vaccines help protect against bacterial infections caused by Streptococcus pneumoniae (pneumococci). Pneumococcal infections include ear infections, sinusitis, pneumonia... read more , influenza Influenza Vaccine The influenza virus vaccine helps protect against influenza. Two types of influenza virus, type A and type B, regularly cause seasonal epidemics of influenza in the United States. There are... read more , and COVID-19 COVID-19 Vaccine Coronavirus disease 2019 (COVID-19) vaccines provide protection against COVID-19. COVID-19 is the disease caused by infection with the SARS-CoV-2 virus. There are multiple COVID-19 vaccines... read more .