Respiratory subtype of relapsing polychondritis frequently presents as difficult asthma: a descriptive study of respiratory involvement in relapsing polychondritis with 13 patients from a single UK centre

Abstract

IntroductionRelapsing 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.MethodsThis 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.ResultsWe 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.ConclusionsRP 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.

Clinical analysis of relapsing polychondritis with airway involvement

“…Prior reports revealed that additional investigations, such as CT and endoscopy of the neck and chest, can be applied for the early diagnosis and differential diagnosis of relapsing polychondritis. [16][17][18] In our study, the rate of positive chest CT examination findings, namely varying degrees of airway thickening, stenosis and calcification, was 88.9 per cent in airway-affected patients. The predominant symptom was thickening of the main bronchial wall, which is concordant with the findings of Wang et al 13 Bronchoscopy exhibited findings that were positive for airway involvement in 85.7 per cent of patients.…”

Treatment of relapsing polychondritis: a systematic review

“…AZA showed overall response rates ranging from 38 to 100% and CYC from 25 to 100%. In the study by Dubey et al (13), the poor outcome observed with CYC may be explained by its use in refractory disease. Too few patients were exposed to other molecules to draw any conclusions.…”

Early Recognition and Treatment of Relapsing Polychondritis

“…The average time from onset to diagnosis has been reported to be 2.9 years, and approximately one-third of patients are reported to have visited more than five doctors before diagnosis [7]. Further, RP can be misdiagnosed as bronchial asthma [8]. There are several case reports where the patients had already developed tracheobronchomalacia at the time of the diagnosis of RP [9,10].…”