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Relapsing polychondritis: a clinical review for rheumatologists
Jack Kingdon1, Joseph Roscamp1, Shirish Sangle2
1School of Medical Education, King's College London, UK.
Rheumatology (Oxford, England)
|November 11, 2017
Summary
Relapsing polychondritis (RPC) is a rare autoimmune disease causing cartilage inflammation. This review guides rheumatologists on diagnosing and managing RPC, focusing on pulmonary involvement and anti-TNF-α therapies.
Area of Science:
- Rheumatology
- Autoimmune Diseases
- Rare Diseases
Background:
- Relapsing polychondritis (RPC) is a rare autoimmune rheumatic disorder.
- Traditionally classified as a systemic vasculitis, it involves cartilage inflammation.
- Key features include chondritis of the nasal bridge, auricular chondritis, ocular inflammation, and bronchial tree involvement.
Purpose of the Study:
- To summarize the clinical features of relapsing polychondritis (RPC).
- To provide guidance for rheumatologists on RPC diagnosis and organ involvement assessment.
- To review current RPC management, emphasizing anti-TNF-α agents for pulmonary involvement.
Main Methods:
- Literature review of relapsing polychondritis (RPC) clinical features.
- Analysis of diagnostic challenges and management strategies.
- Focus on anti-TNF-α agents in RPC patients with pulmonary disease.
Main Results:
- RPC diagnosis is often delayed due to its rarity.
- No conventional management guidelines currently exist for RPC.
- Pulmonary involvement is the leading cause of mortality and morbidity in RPC.
Conclusions:
- Early diagnosis and comprehensive organ assessment are crucial for managing relapsing polychondritis (RPC).
- Anti-TNF-α agents show promise in managing pulmonary involvement, a critical aspect of RPC.
- Further research and guideline development are needed for optimal RPC patient care.
