Disease Directory Relapsing Polychondritis
Immune

Relapsing Polychondritis

Also known as: RP, Meyenburg-Altherr-Uehlinger syndrome, chronic atrophic polychondritis

Prevalence

3.5 in 1,000,000

Onset

Any age; peak in 4th-5th decade

Type

Autoimmune cartilage and proteoglycan disorder

About Relapsing Polychondritis

Relapsing Polychondritis is a rare systemic autoimmune disease characterized by episodic and progressive inflammation of cartilaginous structures throughout the body, including auricular, nasal, laryngotracheal, and articular cartilage, as well as proteoglycan-rich structures of the eye, inner ear, and cardiovascular system. The immunopathogenesis involves autoantibodies against type II collagen and matrilin-1, combined with T-cell mediated cartilage destruction. Without adequate treatment, tracheobronchomalacia from airway cartilage destruction represents the most life-threatening complication.

Common Clinical Features

Painful auricular chondritis with erythema (sparing the ear lobe) Nasal chondritis with saddle-nose deformity Laryngotracheal involvement causing hoarseness and stridor Inflammatory arthritis (non-erosive, seronegative) Episcleritis and scleritis Sensorineural hearing loss and vestibular dysfunction Aortic regurgitation or aneurysm

Clinical Trial Eligibility Tips

What to know before applying to Relapsing Polychondritis trials.

Diagnostic criteria (McAdam or modified Damiani) fulfillment should be documented; biopsy confirmation of chondritis significantly strengthens trial eligibility where pathology is requested

Airway involvement severity (spirometry, CT trachea, bronchoscopy findings) is a key stratification variable in trials; current pulmonary function tests are essential

Concurrent myelodysplastic syndrome occurs in 10-30% of RP patients and may affect eligibility; bone marrow biopsy results should be available if cytopenias are present

Patient Resources

Patient Organization

Relapsing Polychondritis Foundation

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Orphanet

European reference resource for rare diseases (ORPHA:728)

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NORD

National Organization for Rare Disorders

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Find recruiting Relapsing Polychondritis trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Relapsing Polychondritis. Updated daily.

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