Fever and cough without pulmonary abnormalities on CT: relapsing polychondritis restricted to the airways

Laryngotracheal involvement is a major cause of morbidity and mortality.1 An average of 2·9 years elapses from onset to diagnosis, and identifying relapsing polychondritis can be challenging, especially in the absence of typical auricular or nasal involvement.2 When the symptoms are only prolonged fever and cough with no pulmonary abnormalities on CT, the differential diagnosis includes extrapulmonary airway disorders such as sinusitis, relapsing polychondritis, sarcoidosis, and asthma due to eosinophilic granulomatous polyangiitis, intravascular lymphoma, which can be hard to diagnose even on high-resolution CT,3 and large vessel vasculitis.4 Tracheal tenderness is suggestive of relapsing polychondritis1 and our patient had tracheal wall thickening visible on CT, but because relapsing polychondritis restricted to the airways is a rare presentation of a rare disease we did further investigations to exclude other causes.