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What is Polychondritis Syndrome?

Published in Autoimmune Disease 3 mins read

Relapsing polychondritis (RP) is a rare disorder characterized by inflammation and destruction of cartilage in various parts of the body, including the ears, nose, joints, eyes, and respiratory tract. Flares of inflammation come and go, varying in severity and frequency from person to person.

Here's a more detailed look at relapsing polychondritis:

Key Features of Relapsing Polychondritis:

  • Cartilage Inflammation: The hallmark of RP is inflammation of cartilage, the flexible tissue that provides structure and support. This inflammation damages the cartilage, leading to pain, swelling, and eventual destruction.

  • Affected Areas: While any cartilage can be affected, the most common sites include:

    • Ears: Auricular chondritis (inflammation of the ear cartilage) is a very common symptom, causing redness, swelling, and pain in the ear.
    • Nose: Nasal chondritis leads to inflammation and eventual collapse of the nasal cartilage, resulting in a "saddle nose" deformity.
    • Joints: Arthralgia (joint pain) and arthritis (joint inflammation) are common, affecting both small and large joints.
    • Eyes: Inflammation of the eyes (episcleritis, scleritis, uveitis) can cause redness, pain, and vision problems.
    • Respiratory Tract: Tracheal and laryngeal cartilage inflammation can cause breathing difficulties, cough, and hoarseness. This is one of the most serious complications.
  • Relapsing Nature: The disease is characterized by flares or episodes of inflammation, followed by periods of remission. The frequency and severity of these flares vary.

Symptoms of Relapsing Polychondritis:

Symptoms vary depending on the location and severity of the cartilage inflammation. Common symptoms include:

  • Ear pain, redness, and swelling
  • Nasal pain and collapse (saddle nose deformity)
  • Joint pain and swelling
  • Eye inflammation, redness, and pain
  • Cough, hoarseness, and breathing difficulties
  • Fever
  • Fatigue
  • Weight loss

Diagnosis:

Diagnosis can be challenging because RP is rare and its symptoms can overlap with other conditions. There is no single definitive test. Diagnosis typically involves:

  • Physical examination: Assessing the symptoms and signs of cartilage inflammation.
  • Blood tests: Looking for signs of inflammation, such as elevated ESR (erythrocyte sedimentation rate) and CRP (C-reactive protein).
  • Imaging studies: CT scans or MRIs can help visualize cartilage inflammation and damage, especially in the trachea.
  • Biopsy: A cartilage biopsy may be performed to confirm the diagnosis.

Treatment:

There is no cure for RP, but treatment can help control inflammation, relieve symptoms, and prevent complications. Treatment options include:

  • NSAIDs: Nonsteroidal anti-inflammatory drugs for mild symptoms.
  • Corticosteroids: Prednisone and other corticosteroids are often used to reduce inflammation during flares.
  • DMARDs: Disease-modifying antirheumatic drugs, such as methotrexate, may be used to suppress the immune system and prevent flares.
  • Biologic agents: TNF inhibitors (e.g., infliximab, etanercept) and other biologics may be used in severe cases that do not respond to other treatments.

In summary, relapsing polychondritis is a rare autoimmune disorder that inflames and damages cartilage, primarily affecting the ears, nose, joints, eyes, and respiratory tract. Diagnosis relies on clinical findings and supporting tests, and treatment aims to manage inflammation and prevent complications.

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