TY - JOUR T1 - Respiratory subtype of Relapsing Polychondritis (RP) frequently presents as difficult asthma: a descriptive study of respiratory involvement in RP with 13 patients from a single UK centre JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00170-2020 SP - 00170-2020 AU - Shirish Dubey AU - Colin Gelder AU - Grace Pink AU - Asad Ali AU - Christopher Taylor AU - Joanna Shakespeare AU - Susan Townsend AU - Patrick Murphy AU - Nicholas Hart AU - David D'Cruz Y1 - 2021/01/01 UR - http://openres.ersjournals.com/content/early/2020/11/26/23120541.00170-2020.abstract N2 - Introduction Relapsing Polychondritis (RP) is a rare multisystem vasculitis characterised by recurrent cartilage inflammation. Respiratory involvement, of which tracheobronchomalacia (TBM) is the commonest form, is difficult to treat and is linked to increased mortality. We describe 13 patients with respiratory involvement.Methods This is a retrospective study of all the patients with RP at University Hospitals Coventry and Warwickshire NHS Trust (UHCW), a secondary care provider for ∼500 000. Only patients with respiratory involvement were included in this study.Results We identified 13 patients who fulfilled the inclusion criteria. Most patients were identified from the “difficult asthma” clinic. TBM was seen in 11 patients, whilst 2 patients had pleural effusions which resolved with immunosuppression and 1 patient had small airways disease. CT scans (inspiratory and expiratory) and bronchoscopy findings were useful in diagnosing TBM. Pulmonary function testing revealed significant expiratory flow abnormalities. All patients were treated with corticosteroids/disease modifying anti rheumatic drugs (DMARDs) and some were given Cyclophosphamide or biological agents although the response to Cyclophosphamide (1 out of 4) or biologicals (2 out of 4) was modest in this cohort. Ambulatory continuous positive airway pressure (CPAP) ventilation was successful in 4 patients.Conclusions RP may be overlooked in “difficult asthma” clinics with patients having TBM (not asthma) and other features of RP. Awareness of this condition is crucial to enable early diagnosis and interventions to reduce the risk of life threatening airway collapse. A number of patients respond well to DMARDs and are able to minimise corticosteroid use.FootnotesThis manuscript has recently been accepted for publication in the ERJ Open Research. It is published here in its accepted form prior to copyediting and typesetting by our production team. After these production processes are complete and the authors have approved the resulting proofs, the article will move to the latest issue of the ERJOR online. Please open or download the PDF to view this article.Conflict of interest: Dr. Dubey has nothing to disclose.Conflict of interest: Dr. Gelder has nothing to disclose.Conflict of interest: Dr. Pink has nothing to disclose.Conflict of interest: Dr. Ali has nothing to disclose.Conflict of interest: Dr. Taylor has nothing to disclose.Conflict of interest: Dr. Shakespeare has nothing to disclose.Conflict of interest: Susan Townsend has nothing to disclose.Conflict of interest: P. Murphy reports grants paid to his institution and personal fees for CPD approved activity from Philips, ResMed, F&P and B&D Electromedical, advisory board fees from Santhera, and grants paid to his institution from GSK, outside the submitted work.Conflict of interest: N. Hart reports an unrestricted research grant for the OPIP Trial from Philips-Respironics, personal fees for a lecture at TOP Forum China from Philips-Respironics Lecture, and unrestricted research grants for the HoT-HMV Trial from RESMED and Philips-Respironics, outside the submitted work; in addition, he has a European patent issued and a US patent pending for MYOTRACE. His research group has received unrestricted grants (managed by Guy's & St Thomas' Foundation Trust) from Philips and Resmed. Philips are contributing to the development of the MYOTRACE technology.Conflict of interest: Dr. D'Cruz has nothing to disclose. ER -