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Relapsing polychondritis: a clinical review
Erik Letko1, Panayotis Zafirakis, Stefanos Baltatzis
1Uveitis and Immunology Service, Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, MA 02114, USA.
Seminars in Arthritis and Rheumatism
|June 22, 2002
Summary
Relapsing polychondritis (RP) is a rare, progressive autoimmune disease affecting cartilage and other tissues. While treatment options exist, its pathogenesis and optimal management remain poorly understood, impacting patient prognosis.
Area of Science:
- Rheumatology
- Immunology
- Internal Medicine
Background:
- Relapsing polychondritis (RP) is a rare, episodic, and progressive inflammatory condition.
- First described in 1923, RP is presumed to have an autoimmune etiology.
- The disease primarily affects cartilage in various organs and proteoglycan-rich tissues.
Purpose of the Study:
- To comprehensively review the existing literature on relapsing polychondritis (RP).
- To analyze the history, epidemiology, etiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of RP.
Main Methods:
- A systematic literature search was conducted using MEDLINE (PubMed).
- The search term "relapsing polychondritis" was utilized.
- Relevant articles were critically analyzed for key aspects of the disease.
Main Results:
- RP affects cartilage (ear, nose, larynx, trachea, bronchi, joints) and other tissues (eyes, aorta, heart, skin).
- Diagnosis relies on clinical criteria; no standardized treatment protocol exists.
- Treatment involves NSAIDs, dapsone, colchicine, immunosuppressants, or prednisone; survival rates have improved with better management.
Conclusions:
- Relapsing polychondritis is a rare, multisystemic, and potentially fatal disease.
- The underlying pathogenesis and optimal therapeutic strategies for RP require further investigation.