Background/Purpose: To estimate the incidence, prevalence and mortality of Relapsing Polychondritis (RP), and to describe the clinical features of RP, in a large population. 

Methods: All participants diagnosed with RP were sampled from the Clinical Practice Research Datalink (CPRD). Prevalence and incidence rates for 1990-2012 were estimated. Relative mortality of RP cases was estimated in a time-to-event framework using UK life tables for reference. A questionnaire validation study was performed.

Results:  There were 117 participants with RP ever recorded. In the validation study, 47/58 (81%) of cases were confirmed by a physician and unconfirmed cases were excluded. The analysis included 106 participants (42 men, 64 women) diagnosed with RP. The mean age (range) at diagnosis in men was 55 (17 to 81) years and in women 51 (11 to 79) years. There was a median interval of 1.9 years from first symptom consultation to diagnosis. The incidence of RP between 1990 and 2012 was 0.71 (0.55 to 0.91) per million population per year. There were 19 deaths from any cause. There were 16 observed deaths eligible for survival analysis and 7.4 deaths expected for the UK population of the same age, sex and period. The standardised mortality ratio was 2.16 (1.24 to 3.51), p<0.01. Respiratory disease, cardiac conditions and cancer were the most frequent causes of death.

Conclusion: The incidence of RP may be lower than previously estimated and diagnostic misclassification and delay may be frequent. Mortality in RP is more than twice that of the general population.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/incidence-and-mortality-of-relapsing-polychondritis-in-the-united-kingdom-a-population-based-cohort-study/