What is relapsing polychondritis?
Relapsing polychondritis is a rare, long-term disease believed to be autoimmune in nature. This means the immune system mistakenly attacks the body’s own cartilage, leading to repeated episodes of swelling and damage in different parts of the body. The condition most often affects the ears, nose, joints, and airways, but it can also involve the eyes, heart, blood vessels, and skin. The exact cause is unknown, but researchers believe there may be a genetic component along with possible environmental triggers. It’s also sometimes seen in people with other autoimmune disorders, such as lupus or vasculitis. Most cases begin between the ages of 40 and 60.
Penn Medicine offers expert care for a wide range of autoimmune and connective tissue diseases, including relapsing polychondritis.
Relapsing polychondritis symptoms
Symptoms of relapsing polychondritis (RP) can vary depending on which parts of the body are affected. Common signs include fatigue, low fever, and skin rashes, along with more specific symptoms like:
- Pain, redness, and swelling in the outer ear cartilage and a possible floppy or misshapen ear
- Tenderness and swelling in the nose that can lead to a sunken nasal bridge (“saddle nose”)
- Joint pain, swelling, and stiffness, often in the hands, knees, or chest
- Hoarseness, coughing, shortness of breath, or breathing problems due to swelling in the trachea (windpipe) or larynx (voice box)
- Eye pain or redness, or vision changes from inflammation
Diagnosing relapsing polychondritis
A diagnosis of relapsing polychondritis is based on a combination of clinical signs, including inflammation in cartilage-rich areas like the ears, nose, or airways, and symptoms like joint pain or eye inflammation. Doctors use blood test results and imaging to look for signs of inflammation and to rule out other possible causes, such as infectious, cancer-related, or other autoimmune diseases. Removing a small sample of cartilage tissue for examination under a microscope is sometimes done to help confirm the diagnosis.
Treatment for relapsing polychondritis
Treatment for relapsing polychondritis focuses on reducing inflammation, managing symptoms, and preventing damage to affected organs. Most people are treated with medications such as anti-inflammatory drugs, corticosteroids, or immunosuppressants, depending on the severity of the disease. In some cases, especially when the airways or heart are involved, surgery may be necessary to relieve symptoms or repair damage.
Coordinated care for complex autoimmune conditions
Penn Medicine brings together top experts from multiple specialties to care for people with relapsing polychondritis. Experts in rheumatology, pulmonology, radiology, ophthalmology, and ear, nose, and throat (ENT), among others, work together to provide:
- Advanced testing and tools to help diagnose and track the disease
- A team approach that investigates all aspects of the condition
- Individualized care that fits your unique symptoms and needs
As a national leader in autoimmune disease research, we’re working to better understand complex conditions like relapsing polychondritis and to find safer, more effective treatments.