Relapsing Polychondritis

Recurrent, bilateral, painful, red, swollen ears that spare the earlobes, together with a saddle-nose deformity and a seronegative arthritis, is the classic picture of relapsing polychondritis, an immune-mediated disease of recurrent inflammation and destruction of cartilage. The lobe-sparing pattern is the key clue, because the earlobe is non-cartilaginous and is therefore spared, while the cartilaginous pinna is inflamed; recurrent attacks produce a floppy, cauliflower ear. Nasal chondritis gives the saddle-nose deformity, and the arthritis is typically seronegative, non-erosive, episodic and often migratory. I would make the diagnosis clinically using the McAdam or modified Michet criteria, supported by inflammatory markers, and I would remember that classical features can appear late. The main danger is laryngotracheal involvement: inflammation can soften and collapse or stenose the airway, causing hoarseness, stridor and potentially fatal airway obstruction, and it is a leading cause of death, so respiratory symptoms demand urgent airway assessment. I would also screen for the important associations - other autoimmune disease, ANCA-associated vasculitis, and haematological disease including myelodysplastic syndrome and VEXAS in older men. Management is medical and multidisciplinary: corticosteroids for flares with steroid-sparing immunosuppression such as methotrexate or azathioprine, biologics or avacopan in refractory or overlap cases, and urgent airway management when the larynx and trachea are involved.