Background/Purpose: Relapsing polychondritis (RP) is a rare autoimmune disorder characterized by cartilage inflammation and damage. Chronic RP may lead to sequelae due to cartilage tissue damage. There is no diagnostic test for RP and prompt diagnosis remains a challenge due to the marked variation in initial clinical presentation. We describe disease patterns amongst patients with RP, and identify factors that correlate with diagnostic delay and poor outcome.

Methods: This was a retrospective cohort study of RP patients followed up for 20 years in our systemic vasculitis clinic. Patient data was retrieved from June 1995 until 31^stDecember 2015, or until patient’s last follow-up date. Data recorded include patients’ demographic, initial clinical presentations, therapeutic regimes, and long-term outcome. Data analysis was performed using SPSS Version 23.0

Results: 45 patients were identified. 68.9% were female. 39 patients (86.7%) were Caucasian, 5 (11.1%) Afro-Caribbean and 1 (2.2%) Asian. Median age of disease onset was 39.0 (29.5, 54.0) years. Median age of RP diagnosis was 42.0 (34.5, 60.5) years. Median diagnosis delay was 19.0 (5.0, 65.0) months. Most had ear involvement at presentation (73.3%, n=33), followed by chest wall or peripheral joint pain (57.8%), eyes (53.3%), nose (42.2%), and airways (28.9%). Mean number of initial organ presentation was 2.6 ± 1.04. All patients received steroid as therapeutic agent. There was wide heterogeneity in terms of therapeutic agents used, including methotrexate (31.1%), azathioprine (26.7%), hydroxychloroquine (13.3%), mycophenolate (13.3%), and IV cyclophosphamide (11.1%). Mean number of drugs used at any time was 1.9 ± 1.00. Long-term sequelae involved chronic pain (64.4%) and drugs related complications (57.8%). 57.8% had ears damages, followed by eyes damages (42.2%), and airway damages (40%). Mean number of organ damages was 1.7 ± 1.37. Higher number of initial organ presentation (r= 0.34, p= 0.022) and younger age of disease onset (r= -0.40, p= 0.006) correlated with diagnosis delay. Male gender correlated with airway involvement (r= 0.42, p= 0.004). There was positive correlation between airway involvement with higher number of organ damage (r= 0.30, p= 0.047). A weak correlation was noted between initial involvement of chest wall or peripheral joint pain with chronic pain complication (r= 0.34, p= 0.024). 30 patients (66.7%) remained under active follow-up, with 11 patients (24.4%) lost to follow-up, and 4 deaths occurred with cause unknown (8.9%).

Conclusion: Our study revealed higher number of initial organ presentation, and younger age of disease onset correlated with potential diagnosis delay. Male gender with airway involvements correlated with higher number of organ damages and poorer outcome. Multicenter registries may lead to a better understanding of this disease.

« Back to 2016 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/disease-patterns-and-long-term-outcome-amongst-patients-with-relapsing-polychondritis-single-centre-experience/