Background/Purpose:

Relapsing Polychondritis (RP) is a rare immune mediated inflammatory disorder that may result in destruction of cartilaginous tissues.  Diagnostic delay is common due to its heterogeneous clinical spectrum and its rarity. RP is most commonly seen in Caucasians. Pulmonary manifestations are common and are associated with significant morbidity and mortality.

Methods:

We completed a retrospective data analysis of patients attending the Louise Coote Lupus Unit with a clinical diagnosis of RP, focusing on those with respiratory involvement. We used McAdams classification criteria. All patients had lung function tests, high resolution CT scan imaging and bronchoscopy / laryngoscopy wherever necessary along with inflammatory markers and serology to rule out other diseases such as ANCA vasculitis.

Results:

We identified 57 patients with a diagnosis of RP, with respiratory involvement in 23 patients (40%) (14 female and 9 male). 18 patients (78%) were Caucasian, 3 (13%) Afro Caribbean and 2 (9%) Asian. Sixteen (70%) patients presented with respiratory symptoms ranging from asthma like illness to the need for emergency tracheostomy. Median age at the symptom onset varying from 18-70 (median age of 41). There was a mean delay in diagnosis of 82 months.  32/57 patients fulfilled McAdams classification criteria. The other 25 patients had clinical presentations compatible with a diagnosis of RP.

Median ESR was 10 (5-70) mm per 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 and 2 had an emergency tracheostomy.

Most patients were on a combination of oral prednisolone and disease modifying anti-rheumatic drugs.

Four patients received biologics. One received rituximab, two Infliximab and one adalimumab. Two patients did not respond to treatment (rituximab and infliximab). The remaining two patients had a good response.

Five 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.

Conclusion:

Pulmonary involvement in RP may cause significant morbidity and mortality due to organ damage. All RP patients should be evaluated for pulmonary involvement and early detection may help to prevent the damage. Immunosuppressive agents should be considered as soon as the diagnosis of RP with respiratory involvement is established. The role of biologic therapies in treatment resistant patients is uncertain.

Pulmonary complications in RP

MTX = Methotrexate, MMF = Mycophenolate Mofetil, AZA = Azathioprine, HCQ = Hydroxychloroquine