Background/Purpose: Relapsing polychondritis (RP) is a multi-system disease characterised by episodes of progressive inflammation and subsequent degeneration of cartilage and connective tissue throughout the body. RP can be associated with significant morbidity and increased mortality. Respiratory manifestations include large and small airway disease. Tracheobronchomalacia is one of the severe forms of respiratory involvement and can lead to sudden death due to collapse of the airways. RP is a rare condition with incidence of around 1 in a million. We present 11 patients who presented with severe airways disease, and subsequently were diagnosed as relapsing polychondritis from one centre in UK with catchment area of around 500,000.

Methods:

The index case was diagnosed at Louise Coote Unit with RP on the basis of tracheobronchomalacia, bilateral auricular chondritis, nasal chondritis, costochondritis and inflammatory arthritis.

All patients had presented to the difficult airways clinic at University Hospital Coventry and Warwickshire NHS Trust (UHCW) in Coventry with severe breathlessness, cough and monophonic wheeze or been admitted as emergency with severe ‘exacerbation of asthma’. RP was diagnosed using the McAdam criteria) and disease activity assessed using the Relapsing Polychondritis Disease Activity Index (RPDAI). Tracheobronchomalacia was usually initially suspected on clinical examination particularly the presence of fixed inspiratory or harsh monophonic inspiratory and expiratory wheeze. Diagnosis of tracheobronchomalacia was confirmed by a combination of lung function testing, inspiratory and expiratory CT and bronchoscopy.

Results: All patients had three or more of the six diagnostic clinical features described by McAdam et al. (1976). There was considerable delay from the onset of respiratory symptoms to the diagnosis of RP. Flow-volume loops demonstrated flattening of usually expiratory curves (intrathoracic). CT showed obvious dynamic major airway collapse on expiratory films in the majority. Other manifestations of autoimmunity, such as anti-phospholipid syndrome, or other autoimmune conditions were present in 5 patients.

Patients were initially treated with a combination of oral prednisolone and methotrexate or azathioprine. 3 patients were treated with IV Cyclophosphamide, although none responded. Five patients have been treated with continuous positive airway pressure(CPAP) to palliate airway collapse. Anti-TNF treatment was tried in 3 patients (Infliximab in one with good response, Etanercept in another 2 without response). One of these has now been switched to Abatacept, whilst the other is due to start Secukinumab.

Conclusion: Obstructive small or large airways disease is a common presentation for relapsing polychondritis which is contrary to previous reports.

Monophonic wheeze should encourage investigations for tracheobronchomalacia.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/11-patients-with-relapsing-polychondritis-presenting-with-severe-airways-disease-in-one-centre-in-uk/