OBJECTIVES: We developed and tested a robust case ascertainment strategy within the Clinical Practice Research Datalink (CPRD), with the aim of assessing the incidence, prevalence, mortality and delay in diagnosis of SSc in the UK.
METHODS: A two-stage case ascertainment strategy was devised and tested to establish a valid cohort of SSc cases within the CPRD. Incidence, prevalence and mortality statistics were analysed, alongside evaluation of the relationship between primary care codes for RP and SSc to examine diagnostic delay.
RESULTS: SSc Read codes were identified in 3123 patients (from a study cohort of >10.1 million individuals). Of these, 1757 cases of SSc were identified using our case ascertainment approach. The overall incidence rate of SSc over the period between 1999 and 2017 was 10.7/million/year (95% CI: 9.9-11.4), being higher in females [17.69/million/year (95% CI: 16.32-19.07)] than in males [3.59/million/year (95% CI: 2.97-4.21)]. The overall prevalence of SSc in adults was 235.5/million (95% CI: 207.2-245.7). The mean rate of mortality was 32/1000 person-years, with an overall standardized mortality ratio of 3.51 (95% CI: 3.19-3.84). Of those with an initial code of RP prior to a Read code of SSc, 191/854 (22.4%) had a lag period of >10 years.
CONCLUSION: We have developed and tested a robust case ascertainment strategy to examine the incidence, prevalence, mortality and diagnostic delay of SSc using primary care records of over 10 million UK residents. A significant lag between coding of RP and SSc in many patients suggests diagnostic delay in SSc remains an important unmet need.