Relapsing Polychondritis
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Direct answer
A red, exquisitely tender, swollen ear that spares the non-cartilaginous earlobe is the signature opening of relapsing polychondritis — recurrent, immune-mediated inflammation of cartilaginous structures bilaterally, which then declares itself across the nose (saddle deformity), the tracheobronchial tree (collapse and the main cause of death), the joints (non-erosive seronegative polyarthritis), the eyes, and the inner ear (sensorineural hearing loss with vertigo). McAdam's criteria require at least three of six features for a clinical diagnosis, with biopsy support where needed. Treatment runs from NSAIDs for mild flares, through prednisolone 0.5-1 mg/kg for significant disease with methotrexate or mycophenolate as steroid-sparing agents, to biologic therapy for refractory airway disease; a coexisting myelodysplastic syndrome should be sought in older patients.
What you must remember
- McAdam criteria (three or more of six): bilateral auricular chondritis; non-erosive seronegative inflammatory polyarthritis; nasal chondritis; ocular inflammation (conjunctivitis, scleritis, episcleritis, keratitis, uveitis); cochlear or vestibular dysfunction; respiratory tract chondritis.
- Earlobe sparing: the lobule lacks cartilage — its preservation separates chondritis from cellulitis and is the single most quoted bedside clue.
- Airway is the killer: laryngotracheobronchial chondritis causes stridor, collapse and infections; assess with flow-volume loops, dynamic CT and cautious bronchoscopy; stents or tracheostomy may be needed.
- Audiovestibular damage: sudden sensorineural hearing loss, tinnitus and vertigo reflect involvement of the eustachian tube and inner ear — sometimes permanent.
- Cardiovascular signal: aortitis with aortic regurgitation, ascending aneurysms and, rarely, aortic rupture belong to the follow-up echocardiography.
- Associations: myelodysplastic syndrome in up to a tenth of older patients — monitor the full blood count; also vasculitides, systemic lupus erythematosus and inflammatory bowel disease.
- Treatment ladder: NSAIDs for auricular-only disease; prednisolone 0.5-1 mg/kg for flares; methotrexate, azathioprine or mycophenolate for maintenance; biologics for refractory disease; airway intervention as needed.
- Differential anchors: cellulitis (earlobe involved, unilateral, responds to antibiotics), granulomatosis with polyangiitis (destructive nasal disease, ENT bleeding), red ear syndrome (migraine-associated, brief).
A typical exam case
A 46-year-old man has been treated three times at a periphery centre for "cellulitis of both ears" — each episode settling with antibiotics, each time the lobule untouched. Between episodes he developed a hoarse voice and a nasal twang; now he has stridor on climbing stairs and a collapsing nasal bridge. Score him against McAdam: bilateral auricular chondritis, nasal chondritis, and respiratory tract involvement — three criteria, diagnosis made without waiting for the sixth organ. The next steps are prioritised exactly as the disease threatens: pulmonary function with flow-volume loops (fixed upper-airway obstruction pattern), dynamic expiratory CT of the trachea, and gentle bronchoscopy by someone who can manage a critical airway. Start prednisolone 0.5-1 mg/kg with a steroid-sparing agent planned from the outset, because relapsing courses make steroid toxicity the second disease; involve cardiothoracic colleagues early for stenting considerations, and add an annual echocardiogram for aortic regurgitation.
The teaching point to carry: this patient's mortality is decided by airway timing, not by rash or joints. Hoarseness plus exertional stridor in relapsing polychondritis is an emergency assessment, not a routine follow-up symptom.
Where students slip
The classic error is anchoring on the ear: recurrent bilateral auricular swelling gets read as infection or trauma, and the earlobe check — one second of inspection — that would redirect the diagnosis is skipped. The second slip is under-calling the airway: a normal chest radiograph says nothing about tracheobronchial cartilage, so a patient with hoarseness needs flow-volume loops and dynamic CT, not reassurance. Third, students forget the haematology link — a 70-year-old with relapsing polychondritis and macrocytosis should prompt myelodysplasia workup, a viva favourite pairing. In India, where the disease is rare and biopsies are dispatched casually, remember that histology shows perichondrial lymphocytic inflammation but the diagnosis remains clinical; and before committing to long-term immunosuppression, tuberculosis screening is as mandatory here as in any other steroid-dependent illness.
Frequently asked questions
What are the McAdam diagnostic criteria?
At least three of bilateral auricular chondritis, non-erosive seronegative polyarthritis, nasal chondritis, ocular inflammation, cochlear-vestibular dysfunction and respiratory tract chondritis.
Why is the earlobe spared in auricular chondritis?
The lobule contains no cartilage, so inflammation of auricular cartilage respects it — distinguishing relapsing polychondritis from cellulitis.
What is the leading cause of death?
Tracheobronchial chondritis with airway collapse, infection and asphyxiation — hence flow-volume loops and dynamic CT in any symptomatic patient.
What is first-line drug therapy?
NSAIDs for limited auricular disease; prednisolone 0.5-1 mg/kg daily for significant flares, with methotrexate or mycophenolate added for steroid sparing.
Which haematological disease accompanies relapsing polychondritis?
Myelodysplastic syndrome in a proportion of older patients, warranting full blood count and smear surveillance.