Background/Purpose: Systemic Sclerosis (SSc) is an autoimmune disease that is characterised by fibroblast dysfunction and excess extracellular matrix deposition that leads to skin thickening and internal organ damage. Patients with SSc often develop interstitial lung disease (ILD) which is currently the leading cause of death. The burden of SSc in the UK is currently unknown. However, it is reported that a high proportion of patients present with advanced disease at diagnosis. The reasons for late diagnosis in SSc are not fully understood, although different routes to diagnosis have been associated with differences in survival. This study aimed to investigate patient characteristics related to first SSc diagnosis in hospital, compared to primary care.

Methods: We carried out a population-based cross-sectional study using data from the Clinical Practice Research Datalink (CPRD) linked to Hospital Episode Statistics (HES). All adult patients with a diagnosis of SSc were identified using International Classification of Diseases-10-Clinical Modification diagnosis codes in hospital records and READ codes in primary care electronic records combined with an adapted version of the EULAR algorithm for defining SSc. Patients were classified into 3 groups: incident SSc, SSc with incident ILD (SSc-ILD), and SSc with other organ involvement (SSc-OOI). Source of diagnosis and patient characteristics were then described.

Results: The study included 606 individuals with incident diagnosis of SSc recorded in either CPRD or HES. Only 1% of patients had a diagnosis code in both primary care and secondary care records. 81% of the cohort were women and the average age at diagnosis was 61 (SD = 15). 20% of the cohort had SSc-ILD and 63% had SSc-OOI with an overlap of 15%. Patients first diagnosed with SSc in hospital presented higher rates of hospital visits and had a greater proportion of “red-flag” symptoms predictive of SSc (e.g. Raynaud’s or gastrointestinal reflux) prior to SSc diagnosis. Patients with an overlap of SSc-ILD and SSc-OOI tended to be overweight or obese.

Conclusion: Our results provide evidence of differences between patients diagnosed with SSc through UK primary care or secondary/tertiary care routes. These findings should inform the development of initiatives to improve earlier diagnosis of SSc.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/diagnosis-of-systemic-sclerosis-in-the-united-kingdom-an-observational-study-using-the-clinical-practice-research-datalink/