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Relapsing polychondritis.

Bruce A Staats1, James P Utz, Clement J Michet

  • 1Mayo Clinic and Foundation, Division of Pulmonary and Critical Care, Rochester, Minnesota 55905, USA. staats.bruce@mayo.edu

Seminars in Respiratory and Critical Care Medicine
|August 10, 2005
PubMed
Summary
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Relapsing polychondritis (RP) is a rare connective tissue disease. It causes inflammation and cartilage destruction, frequently affecting airways and leading to potential obstruction.

Area of Science:

  • Rheumatology
  • Pulmonology
  • Rare Diseases

Background:

  • Relapsing polychondritis (RP) is a rare multisystemic inflammatory disorder.
  • It is characterized by recurrent inflammation and destruction of cartilaginous structures.
  • RP lacks specific diagnostic laboratory markers, relying heavily on clinical manifestations.

Purpose of the Study:

  • To summarize the key clinical and diagnostic features of relapsing polychondritis.
  • To highlight the significant impact of RP on airway structures and function.
  • To review current therapeutic approaches and their limitations.

Main Methods:

  • Review of existing literature on relapsing polychondritis.
  • Analysis of predominant clinical manifestations and diagnostic challenges.

Related Experiment Videos

  • Evaluation of airway involvement and imaging techniques like CT scans.
  • Assessment of current treatment strategies, including corticosteroids, NSAIDs, and surgical interventions.

    • Main Results:

    • Commonly affects auricular cartilage, joints, nasal cartilage, eyes, and respiratory tract.
    • Airway involvement occurs in 50% of patients, presenting with symptoms like dyspnea and stridor.
    • Tracheobronchial involvement can lead to airflow obstruction due to tracheal collapse, visualized by dynamic CT.
    • No randomized controlled trials exist; treatment relies on corticosteroids and NSAIDs, with optimal regimens undefined.

    Conclusions:

    • Relapsing polychondritis is a rare, relapsing inflammatory disease affecting cartilage.
    • Airway compromise, particularly tracheal collapse, is a critical and potentially life-threatening manifestation.
    • Management is challenging due to lack of evidence-based guidelines, often requiring multidisciplinary approaches.