REFRACTORY RELAPSING POLYCHONDRITIS: WHAT SHOULD A PULMONOLOGIST DO?

Abstract

SESSION TITLE: Fellows Pulmonary Manifestations of Systemic Disease Posters SESSION TYPE: Fellow Case Report Posters PRESENTED ON: October 18-21, 2020 INTRODUCTION: Relapsing polychondritis (RP) is a rare systemic disease characterized by recurrent episodes of inflammation, which can result in degeneration and deformation of the cartilage of the ears, nose, larynx, and tracheobronchial tree. The incidence of RP is 0.7-4.5 per million people with a 20-50% prevalence of respiratory impairment. Airway involvement portends a poor prognosis in this disease, and reporting of cases is beneficial to educate pulmonologists about the importance of a multidisciplinary team in managing patients with RP. CASE PRESENTATION: A 23-year-old female with relapsing polychondritis, subglottic stenosis, and tracheal stenosis status post tracheostomy on chronic immunosuppression with dapsone, mycophenolate, and prednisone presented with worsening shortness of breath despite steroid injections for subglottic stenosis. CT chest demonstrated tracheal narrowing with significant wall thickening of the bilateral mainstem bronchi. She underwent bronchoscopy, which demonstrated erythema in the trachea with loss of the cartilaginous rings, mucosal thickening extending to the bilateral mainstem bronchi including LC2 and RC1, and normal distal bronchi. Even with PEEP, she had complete collapse of the bilateral mainstem bronchi with expiration. Her prednisone dose was increased, but her shortness of breath persisted. Her FEV1 was 46% predicted and FEV1/FVC was 49% predicted on pulmonary function testing. Due to worsening dyspnea at rest, she underwent repeat bronchoscopy 3 months later which demonstrated slightly worsening of inflammation in her mainstem bronchi. She was started on Actemra by rheumatology with subjective improvement in her shortness of breath within 3 weeks. Polysomnography was ordered to try to qualify her for nocturnal non-invasive ventilation. After discussion with otolaryngology, it was decided to not pursue further bronchoscopy for airway evaluation due to concern that bronchoscopy would cause worsening of inflammation of her airways over time. DISCUSSION: Management of refractory RP is challenging for the pulmonologist because there are not evidence based guidelines to follow. Based on expert opinion, pulmonary function testing with flow volume loops is recommended in all patients with RP to characterize airway involvement in symptomatic patients. For the treatment of tracheomalacia, intralesional corticosteroid injections and tracheostomy can be warranted. Tracheobronchial stenting can be helpful, but it also risks worsening of airway inflammation. Thus, 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. CONCLUSIONS: Relapsing polychondritis is a rare disease which is challenging to treat due to a lack of evidence based guidelines. Leveraging the expertise of otolaryngology, pulmonology, and rheumatology is helpful to guide symptomatic management. Reference #1: Lekpa FK, Chevalier X. Refractory relapsing polychondritis: challenges and solutions. Open Access Rheumatol. 2018;10:1-11. Published 2018 Jan 9. doi:10.2147/OARRR.S142892 Reference #2: Ernst A, Rafeq S, Boiselle P, et al. Relapsing polychondritis and airway involvement. Chest. 2009;135(4):1024-1030. doi:10.1378/chest.08-1180 Reference #3: de Montmollin N, Dusser D, Lorut C, et al. Tracheobronchial involvement of relapsing polychondritis. Autoimmun Rev. 2019;18(9):102353. doi:10.1016/j.autrev.2019.102353 DISCLOSURES: No relevant relationships by Maureen McElligott, source=Web Response