Case Report: A Pulmonologist’s Role in Refractory Relapsing Polychondritis

A case report presented at the CHEST Annual Meeting 2020 highlighted an example of a multidisciplinary team approach to managing a patient with relapsing polychondritis (RP).

The patient was a 23-year-old female with RP, subglottic stenosis, and tracheal stenosis status post-tracheostomy on chronic immunosuppression. She received steroid injections for subglottic stenosis but was still experiencing shortness of breath. A CT scan revealed tracheal narrowing with significant wall thickening of the bilateral mainstem bronchi. Bronchoscopy unearthed erythema in the trachea with loss of the cartilaginous rings and mucosal thickening extending to the bilateral mainstem bronchi, with normal distal bronchi. Increased prednisone did not resolve her shortness of breath.

Three months later, bronchoscopy was performed again. This time, the test revealed worse mainstem bronchi inflammation. Rheumatology recommended she begin tocilizumab. Three weeks later, the patient’s shortness of breath improved. Polysomnography was performed to determine the use of nocturnal non-invasive ventilation. Following consultation with otolaryngology, the decision was made to stop the use of bronchoscopy to prevent further inflammation to the patient’s airways.

Given the potential complexities associated with RP, the researchers said, “A team-based approach is helpful to weigh the risks and benefits of treatment options to determine the best care for the patient with worsening symptoms.”

“Leveraging the expertise of otolaryngology, pulmonology, and rheumatology is helpful to guide symptomatic management,” they concluded.