What Is Relapsing Polychondritis?

Relapsing polychondritis (RP) — Systemic chondromalacia or Atrophic polychondritis — is a rare disorder that causes inflammation of the connective tissue, specifically the cartilage surrounding the eyes, nose, and ears. Pain at the cartilage sites and joints is one of the main symptoms of relapsing polychondritis. Lab tests and medical observations help to detect cartilage deterioration, and treatment often involves a prescription of corticosteroids or immunosuppressant medications. The causes of the cartilage deterioration remain unknown. The disease is also known as von Meyenburg's disease or Meyenburg-Altherr-Uehlinger syndrome, as well as chronic atrophic polychondritis and generalized or systemic chondromalacia.

Among the main symptoms of relapsing polychondritis includes pain that occurs suddenly around the ears, nose, and eyes, as well as the joints and tissues. Inflammation is another common symptom that causes stiffness, or arthritis, in the hands and feet, wrists, knees, and ankles. If the swelling occurs in the eyes, then the disorder can lead to keratitis or conjunctivitis. Inflammation in the ears and nose causes “floppy ears” and “saddle nose” deformities because relapsing polychondritis weakens the cartilage. Inflammation around the trachea not only creates breathing problems, but it causes chronic hoarseness and throat pain.

This connective tissue disease can occur in both men and women, and signs often appear during middle age. Primarily, the disorder causes recurring episodes of cartilage and tissue inflammation. Besides inflammation in the nose, eyes, and ears, the disorder targets the spine and joints, which usually leads to arthritis. It also affects the trachea, which endangers breathing. Relapsing polychondritis can also inflame the blood vessels, heart, and kidneys, making the disease life-threatening if not treated promptly.

Inflammation of the heart, blood vessels, and the skin from relapsing polychondritis acts as a catalyst to other issues, such as an aneurysm, pericarditis, or vasculitis. Like some other chronic conditions, relapsing polychondritis also causes weight loss and fatigue. Symptoms range from mild to severe, and they usually last for several weeks.

Causes of relapsing polychondritis have yet to be determined. Doctors have linked the condition to rheumatoid arthritis and other autoimmune diseases because they share similar symptoms. In the case of an autoimmune disease, the body’s immune system experiences trouble with fighting off infections, often leading to inflammation of the tissues.

To diagnose the problem, the doctor first observes the patient for signs of inflammation in the eyes, ears, and other tissues and joints. If the diagnosis remains unclear, then the doctor orders a biopsy of the affected cartilage for further examination. Blood tests also help to detect symptoms of the disorder. The doctor may also use a chest Computed Tomography (CT) scan or spirometric testing to examine for swelling in the trachea and surrounding tissues.

Once testing confirms a diagnosis, treatment often includes regular use of corticosteroids or NSAIDs to reduce inflammation. Immunosuppressant medications may be prescribed for more serious cases. The patient should seek constant long-term medical care to keep symptoms from becoming life-threatening.