What is Relapsing Polychondritis?
Relapsing polychondritis is a rare, chronic autoimmune disease characterized by recurrent inflammation of cartilage and other tissues throughout the body. The condition most commonly affects the ears, nose, joints, and respiratory tract, but it can also impact the eyes, heart, blood vessels, and kidneys in more severe cases.
Causes of Relapsing Polychondritis
The exact cause of relapsing polychondritis is unknown, but it is believed to be an autoimmune disorder, where the immune system mistakenly attacks the body’s own cartilage (a type of connective tissue that is found throughout the body). It can also be associated with other autoimmune diseases such as lupus, rheumatoid arthritis, or vasculitis.
Autoimmune disorders are conditions in which the body’s immune system mistakenly attacks its own healthy tissue. Generally, the immune system is designed to protect the body from harmful invaders, such as viruses and bacteria, but in autoimmune conditions, the immune system becomes confused and views the body’s healthy tissues as an invader.
Signs and Symptoms of Relapsing Polychondritis
The signs and symptoms of relapsing polychondritis vary widely depending on which areas of the body is affected. It is a relapsing-remitting disease, meaning symptoms come and go, often unpredictably. The most common signs and symptoms by body system include:
- Ears: Red, painful, swollen outer ears (but not the earlobes); recurrent episodes can lead to floppy ears or cauliflower ear deformity
- Nose: Tender, red nose; repeated episodes can cause a saddle-nose deformity
- Joints: Non-erosive arthritis affecting joints like knees, wrists, or ankles
- Eyes: Redness, pain, blurred vision from conditions like scleritis or uveitis
- Respiratory: Hoarseness, cough, or difficulty breathing due to airway cartilage involvement
- Hearing and balance: Hearing loss, dizziness, or tinnitus
- Heart/Vessels: Aneurysms, valve disease, pericarditis
- General symptoms: Fatigue, fever, and weight loss
Diagnosis of Relapsing Polychondritis
The diagnosis of relapsing polychondritis is primarily clinical, based on the presence of characteristic symptoms such as recurrent inflammation of cartilage in the ears, nose, joints, eyes, and respiratory tract. There is no single definitive test, so physicians often rely on clinical criteria like McAdam’s or Damiani’s criteria, which outline specific features of the disease. Blood tests may be performed to detect signs of systemic inflammation (elevated ESR or CRP) and to rule out other autoimmune or infectious conditions. Imaging studies such as CT or MRI can help assess airway or joint involvement, especially if symptoms are severe. In some cases, a biopsy of affected cartilage may be done to confirm inflammation and aid in diagnosis.
Treatment for Relapsing Polychondritis
Treatment for relapsing polychondritis focuses on reducing inflammation and managing symptoms based on disease severity. Mild cases, such as those involving the ears, nose, or joints, are typically treated with nonsteroidal anti-inflammatory drugs (NSAIDs) and low-dose corticosteroids. Moderate cases, involving more widespread inflammation, may require higher doses of corticosteroids along with immunosuppressive medications like methotrexate or azathioprine to control the disease and minimize steroid use. In severe or life-threatening situations, such as airway involvement or organ complications, treatment may involve high-dose intravenous corticosteroids, stronger immunosuppressants like cyclophosphamide, or biologic therapies such as rituximab or TNF inhibitors. Supportive care, including airway interventions, eye treatments, and hearing aids, may also be necessary depending on the organs affected. Treatment is tailored to the individual based on the severity and organs involved, and regular monitoring is important to manage flares and adjust medications as needed.
