Rare systemic autoimmune disease with recurrent inflammation and progressive destruction of cartilaginous tissues (ears, nose, larynx, etc).
Life-threatening if airway cartilage is involved (tracheomalacia can cause fatal collapse). Classic for exams due to its unique features (e.g., saddle-nose deformity, red ear sparing the lobule) and overlap with other autoimmune diseases.
Typically middle-aged adults (~40–50s). Many cases are associated with other autoimmune disorders (e.g., RA, SLE) or hematologic disease (e.g., myelodysplastic syndrome).
Recurrent auricular chondritis: painful, red, swollen pinnae (outer ears) with earlobes spared. Nasal cartilage inflammation (in ~25%) causes a tender nose and saddle-nose deformity over time.
Laryngotracheal chondritis: presents with cough, hoarseness, wheezing or stridor; can lead to airway collapse (tracheomalacia). Also can see ocular inflammation (episcleritis, uveitis) and a migratory, non-erosive arthritis (often of hands/wrists).
Diagnosis is clinical—suspect RP in anyone with unexplained recurring chondritis of the ear, nose, or airway. Rule out mimics: check ANCA (negative in RP, positive in GPA) and consider infectious causes (e.g., Pseudomonas auricular perichondritis after piercings).
No specific lab test confirms RP. Inflammatory markers may be elevated, but autoantibodies (e.g., ANCA, RF) are often absent. Cartilage biopsy (ear or nose) can provide histologic confirmation if needed (shows cartilage inflammation and loss).
Assess disease extent once diagnosed: always evaluate for airway involvement (e.g., CT scan of trachea) even if no respiratory symptoms. Pulmonary function tests can help quantify fixed airway obstruction from tracheomalacia.
If airway compromise is present or impending (stridor), secure the airway (ENT consult for possible stenting or tracheostomy).
Wegener's granulomatosis – can also cause saddle-nose deformity and airway issues, but usually has lung/kidney involvement and c-ANCA positivity.
Auricular perichondritis (infection)
Cartilage infection (often *Pseudomonas* after trauma/piercing) – typically unilateral and includes the lobule; systemic signs of infection usually present.
Chronic symmetric polyarthritis with positive RF/anti-CCP – can cause scleritis but does NOT inflame auricular cartilage or cause saddle-nose.
Mild cases (ear, nose, joints only): NSAIDs or meds like colchicine/dapsone for symptom control, plus typically low-dose corticosteroids to suppress inflammation.
Severe or progressive cases (airway, eye, inner ear involved): high-dose glucocorticoids (e.g., IV methylprednisolone pulses) combined with immunosuppressants (e.g., cyclophosphamide, methotrexate, or azathioprine) to induce remission.
Refractory cases: biologic agents (most often TNF inhibitors like infliximab) have been used. Ensure airway protection – significant tracheal collapse may require stenting or tracheostomy.
Mnemonic POLY for relapsing polychondritis: Painful Pinnae, Obstructed Airway, Laryngeal/nasal cartilage collapse, Years of relapsing episodes.
Earlobe sparing: inflammation of the external ear in RP involves only the cartilaginous pinna, with the soft lobe remaining normal – a clue distinguishing it from cellulitis.
Stridor or respiratory distress in RP – indicates impending airway collapse; requires emergency airway management (prepare for intubation or surgical airway).
Severe ocular involvement (scleritis/uveitis) or sudden hearing loss – sign of aggressive disease; treat promptly with high-dose steroids to prevent permanent damage.
If ≥3 of the characteristic features (ear, nasal, laryngotracheal chondritis, ocular inflammation, hearing loss, or seronegative arthritis) are present, diagnosis is likely. Consider confirming with cartilage biopsy if uncertainty.
Evaluate extent of disease (especially airway): perform chest CT or bronchoscopy if breathing symptoms to check for tracheal involvement.
Begin therapy based on severity: e.g., steroids ± immunosuppressants for active disease; urgent ENT intervention if airway is compromised.
Middle-aged patient with recurrent red, swollen ear (lobule spared) and new saddle-nose deformity → relapsing polychondritis.
Patient with long-standing RA who develops a painful auricle and stridor → suspect relapsing polychondritis overlapping (RA alone doesn't cause auricular chondritis).
Case 1
A 52‑year‑old man with recurrent episodes of a painful, swollen right ear now has 2 days of hoarse voice and difficulty breathing.
Ear inflammation sparing the ear lobe in relapsing polychondritis.
