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Relapsing polychondritis: Best Practice & Clinical Rheumatology
Phillip Mertz1, Joshua Sparks2, Dale Kobrin3
1Hôpitaux Universitaires de Strasbourg, Centre National de Référence RESO-Lupus, 67000 Strasbourg, France.
Best Practice & Research. Clinical Rheumatology
|October 15, 2023
Summary
Relapsing polychondritis (RP) is a rare inflammatory disease affecting cartilage. Recent discoveries, like VEXAS syndrome, suggest RP may be multiple conditions, complicating diagnosis and management.
Area of Science:
- Rheumatology and Immunology
- Genetics and Molecular Biology
Background:
- Relapsing polychondritis (RP) is a rare inflammatory condition primarily affecting cartilage.
- It impacts various organs, leading to diagnostic delays due to fluctuating symptoms and lack of specific markers.
- Recent research identifies VEXAS syndrome as a cause for a subset of RP patients, indicating RP may encompass multiple distinct diseases.
Purpose of the Study:
- To provide clinicians with insights for improved diagnosis and management of relapsing polychondritis.
- To highlight the evolving understanding of RP, including its categorization into clinical groups and the impact of new genetic discoveries like VEXAS syndrome.
Main Methods:
- Literature review of relapsing polychondritis and VEXAS syndrome.
- Analysis of clinical manifestations and diagnostic challenges in RP.
- Synthesis of recent findings on genetic causes and disease classification.
Main Results:
- RP affects diverse organs including cartilage, blood vessels, skin, eyes, and inner ear.
- VEXAS syndrome, caused by UBA1 gene mutations, accounts for 8% of RP cases, particularly those with a hematologic subgroup.
- Current diagnostic and management strategies for RP are hampered by symptom variability and lack of standardized criteria.
Conclusions:
- Relapsing polychondritis is likely an umbrella term for multiple distinct diseases, including VEXAS syndrome.
- Accurate diagnosis requires recognizing specific clinical patterns and understanding emerging genetic links.
- Further research is needed to develop standardized classification and severity metrics for better patient care.
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