Respiratory Involvement in Relapsing Polychondritis- a single centre study
Catherine D Hughes, Begona Lopez Garcia, Ken Chee Cheah, Yih JIa Poh, Shirish R Sangle, David D'Cruz
The Louise Coote Lupus Unit, Guy's and St Thomas' Hospital, London UK
Relapsing polychondritis (RP) is a rare immune mediated inflammatory condition that may result in destruction of cartilaginous tissues. Diagnostic delay is common due to its rarity and heterogeneous clinical spectrum.
Pulmonary manifestations are common and are associated with significant morbidity and mortality.
Retrospective data analysis of patients attending the Louise Coote Lupus Unit with a clinical diagnosis of RP, focused on those with respiratory involvement. We used McAdams classification criteria. All patients had lung function tests, high resolution CT imaging and bronchoscopy / laryngoscopy wherever necessary plus inflammatory markers and serology to exclude illnesses such as ANCA positive vasculitis.
We identified 57 patients with a diagnosis of RP, respiratory involvement in 23 patients(40%). 18(78%) patients were Caucasian, 3(13%) Afro-Caribbean and 2(9%) Asian. 16(70%) patients presented with respiratory symptoms ranging from mild asthma-like illness to requiring emergency tracheostomy. Median age at symptom onset varied from 18-70 (median age of 41). Mean diagnostic delay of 82 months. 32/57 patients fulfilled McAdams classification criteria. The other 25 patients had clinical presentations compatible with RP. Median ESR was 10(5-70) mm/hour and CRP was 6(1- 110) mg/l. Respiratory complications: 6 patients had tracheomalacia, 5 had tracheal stenosis +/- thickening, 8 had tracheal and bronchial collapse +/- stents,2 had an emergency tracheostomy. Most patients were on a combination of oral prednisolone (PRED) and disease modifying anti-rheumatic drugs (DMARDs). 4 patients received biologics (1= Rituximab, 2=Infliximab, 1=Adalimumab). 2 patients did not respond to treatment (Rituximab and Infliximab). 2 patients had a good response. 5 patients required CPAP to maintain airways patency due to respiratory collapse. Number of other organ involvement: 7/23 eyes 12/23 ears, 7/23 nose, 17/23 airways, 14/23 chest wall/joints One patient had 5 organ involvement, three had 4 organ involvement, six had 3 organ involvement, nine patients had 2 organ involvement and four patients had only respiratory involvement.
All RP patients should be evaluated for pulmonary involvement and early detection may help to prevent damage. Immunosuppressive agents should be considered as soon as the diagnosis of RP with respiratory involvement is established. The role of biologic therapy in treatment resistant patients is uncertain.