Summary about Disease
Relapsing polychondritis (RP) is a rare autoimmune disease characterized by recurrent inflammation and destruction of cartilage in various parts of the body. This inflammation most commonly affects the cartilage of the ears, nose, trachea, and joints, but can also involve the eyes, heart, blood vessels, and skin. The condition is characterized by periods of active inflammation ("flares" or "outbreaks") followed by periods of remission. The long-term prognosis varies depending on the severity and extent of organ involvement, but it can lead to significant morbidity and, in some cases, mortality.
Symptoms
Symptoms of relapsing polychondritis vary widely and can affect multiple organ systems. Common symptoms include:
Ear: Ear pain, redness, swelling, tenderness. Often affects the ear cartilage but spares the earlobe. Hearing loss, dizziness, and balance problems can also occur.
Nose: Nasal cartilage inflammation, leading to pain, tenderness, and potentially nasal collapse ("saddle nose deformity").
Trachea and Larynx: Inflammation of the airway cartilage, causing hoarseness, cough, difficulty breathing (stridor), and a feeling of choking. This can be life-threatening.
Joints: Joint pain, swelling, and stiffness (arthritis), affecting both large and small joints. Often migratory, meaning it moves from one joint to another.
Eyes: Redness, pain, blurred vision, double vision, conjunctivitis, scleritis, uveitis.
Cardiovascular: Inflammation of the heart valves (aortic insufficiency), aorta (aortitis), or other blood vessels (vasculitis).
Skin: Skin rashes, nodules, or ulcers.
Other: Fever, fatigue, weight loss.
Causes
The exact cause of relapsing polychondritis is unknown. It is considered an autoimmune disorder, meaning the body's immune system mistakenly attacks its own cartilage and other tissues. Genetic factors may play a role, but a specific gene or genetic mutation has not been identified. Environmental triggers may also contribute to the development of the disease in susceptible individuals.
Medicine Used
Treatment for relapsing polychondritis aims to reduce inflammation, control symptoms, and prevent organ damage. Medications used include:
Nonsteroidal anti-inflammatory drugs (NSAIDs): For mild symptoms like joint pain.
Corticosteroids (e.g., prednisone): To quickly reduce inflammation during flares.
Disease-modifying antirheumatic drugs (DMARDs): Such as methotrexate, sulfasalazine, or azathioprine, to suppress the immune system and prevent flares.
Biologic agents: Such as TNF inhibitors (e.g., infliximab, etanercept, adalimumab) or other biologics like tocilizumab, which target specific parts of the immune system.
Dapsone: An antibiotic with anti-inflammatory properties, sometimes used for milder cases.
Other Immunosuppressants: Cyclophosphamide, cyclosporine, and other powerful immunosuppressants may be used in severe cases with organ involvement. The choice of medication depends on the severity of the disease and the organs affected.
Is Communicable
Relapsing polychondritis is not communicable. It is not contagious and cannot be spread from person to person.
Precautions
There are no specific precautions to prevent relapsing polychondritis, as the cause is unknown and not related to external factors like infections. However, individuals diagnosed with RP can take certain precautions to manage their condition:
Adhere to treatment plans: Closely follow the medication regimen prescribed by the doctor.
Avoid triggers: If specific factors seem to trigger flares (e.g., stress, certain foods), try to avoid them.
Protect airways: If airway involvement is present, be aware of signs of respiratory distress and seek immediate medical attention if breathing problems develop.
Protect joints: Use assistive devices if needed to reduce stress on affected joints.
Eye care: Regular eye exams are essential, especially if eye involvement is present.
Monitor cardiovascular health: Watch for signs of cardiovascular problems, such as chest pain or shortness of breath, and inform the doctor.
Manage stress: Stress can exacerbate symptoms, so implement stress-reduction techniques.
Vaccination: Consult with the doctor about appropriate vaccinations, as some immunosuppressant medications may increase the risk of infections.
How long does an outbreak last?
7. How Long Does An Outbreak Last? The duration of an outbreak (flare) in relapsing polychondritis varies significantly from person to person. Some flares may last only a few days or weeks, while others can persist for months. The length of the flare depends on factors such as the severity of the inflammation, the organs involved, and the effectiveness of treatment. With appropriate treatment, it may be possible to shorten the duration and severity of flares.
How is it diagnosed?
Diagnosing relapsing polychondritis can be challenging because the symptoms are variable and can mimic other conditions. There is no single definitive test for RP. Diagnosis is typically based on a combination of:
Clinical findings: Evaluating the patient's symptoms and physical examination findings, such as ear inflammation, nasal cartilage inflammation, and respiratory problems.
Blood tests: Elevated inflammatory markers, such as erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), may be present. Other blood tests may be done to rule out other conditions.
Imaging studies: CT scans or MRI scans of the chest, trachea, and other affected areas can help identify cartilage inflammation and structural changes.
Biopsy: In some cases, a biopsy of affected cartilage (e.g., ear cartilage) may be performed to confirm the diagnosis. However, biopsies are not always necessary and can be difficult to obtain.
Exclusion of other conditions: Ruling out other conditions that can cause similar symptoms, such as infections, vasculitis, and other autoimmune diseases. Diagnostic criteria have been proposed by different groups of experts, but the diagnosis often relies on a physician's clinical judgment based on the overall picture.
Timeline of Symptoms
The timeline of symptoms in relapsing polychondritis is highly variable and unpredictable. The disease is characterized by periods of flares (active inflammation) followed by remissions (periods with little or no symptoms).
Onset: The onset of RP can occur at any age, but it is most common in middle adulthood.
Initial Symptoms: Initial symptoms vary, but often include ear inflammation, joint pain, or respiratory symptoms.
Progression: The disease can progress differently in different individuals. Some people may experience infrequent flares with relatively mild symptoms, while others may have frequent and severe flares with progressive organ damage.
Late-Stage: In late-stage RP, significant cartilage destruction and organ damage can occur, leading to deformities (e.g., saddle nose), respiratory problems, cardiovascular complications, and vision loss. The course of the disease can be chronic and relapsing-remitting, with periods of improvement and worsening over many years. Early diagnosis and treatment can help slow the progression of the disease and prevent complications.
Important Considerations
Early diagnosis is crucial: Early diagnosis and treatment can help prevent or minimize organ damage.
Multidisciplinary care: Management of RP often requires a team of specialists, including rheumatologists, pulmonologists, ENT specialists, ophthalmologists, and cardiologists.
Airway involvement: Airway involvement is a serious complication that can be life-threatening. Patients with RP should be closely monitored for signs of respiratory distress.
Cardiovascular complications: RP can affect the heart and blood vessels, increasing the risk of aortic aneurysms, valve disease, and other cardiovascular problems.
Long-term follow-up: Patients with RP require long-term follow-up to monitor disease activity, assess organ function, and adjust treatment as needed.
Support groups: Joining a support group can provide emotional support and information for patients and their families.
Research: Ongoing research is aimed at better understanding the cause of RP and developing more effective treatments.