Objective To identify the clinical characteristics, treatment, and prognosis of relapsing polychondritis patients with airway involvement. Methods Twenty-eight patients with relapsing polychondritis, hospitalised in the First Hospital of Shanxi Medical University between April 2011 and April 2021, were retrospectively analysed. Results Fifty per cent of relapsing polychondritis patients with airway involvement had a lower risk of ear and ocular involvement. Relapsing polychondritis patients with airway involvement had a longer time-to-diagnosis (p < 0.001), a poorer outcome following glucocorticoid combined with immunosuppressant treatment (p = 0.004), and a higher recurrence rate than those without airway involvement (p = 0.004). The rates of positive findings on chest computed tomography and bronchoscopy in relapsing polychondritis patients with airway involvement were 88.9 per cent and 85.7 per cent, respectively. Laryngoscopy analysis showed that 66.7 per cent of relapsing polychondritis patients had varying degrees of mucosal lesions. Conclusion For relapsing polychondritis patients with airway involvement, drug treatment should be combined with local airway management.

BACKGROUND Relapsing polychondritis is a rare multisystem autoimmune disease that mainly involves systemic cartilage and proteoglycan-rich tissues. If the larynx and trachea are involved, the patient’s condition deteriorates rapidly. When relapsing polychondritis becomes more advanced, the airways collapse and treatment is difficult, rendering a poor prognosis. Therefore, the diagnosis method, treatment strategy and prognosis of relapsing polychondritis with larynx and trachea involvement need to be elucidated to improve clinicians’ awareness of the disease. CASE SUMMARY A man and a woman were admitted because of breathlessness. Relapsing polychondritis was diagnosed after a series of accessory examinations. They were both treated with glucocorticoids and immunosuppressants, and underwent tracheotomy as their breathing difficulties could not be relieved by the medication. CONCLUSION The two cases highlight the importance of the timely diagnosis, full evaluation and initiating individualized treatment of relapsing polychondritis with larynx and trachea involvement. Laryngoscopy, bronchoscopy and pathological examination are helpful in diagnosis of this disease.

Two patients with breathlessness were diagnosed as RP, both of them were successfully diagnosed and were controlled well. As a rare autoimmune multisystem disease, RP should be taken into consideration in patients with dyspnea of unknown cause and should be treated timely.