The incidence, prevalence, and survival of systemic sclerosis in the UK Clinical Practice Research Datalink

Royle, Jeremy; Lanyon, Peter; Grainge, Matthew J.; Abhishek, Abhishek; Pearce, Fiona

doi:10.1007/s10067-018-4182-3

Cited by 78 publications

(44 citation statements)

References 34 publications

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“…As compared with adults, children with jSSc have overall better outcomes, related to a lower frequency of major visceral organ involvement and lower mortality (6, 13, 17). During follow-up, interstitial lung involvement, gastroesophageal dysmotility, and renal involvement are significantly more common in adults, while arthritis and muscle inflammation are more common in children, because of the higher prevalence of the overlap form of jSSc (3, 4, 6).…”

Section: Clinical Aspectsmentioning

confidence: 99%

Immunopathogenesis of Juvenile Systemic Sclerosis

Stevens

Torok

et al. 2019

Front. Immunol.

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Juvenile-onset systemic sclerosis (jSSc) is a rare and severe autoimmune disease with associated life-threatening organ inflammation and evidence of fibrosis. The organ manifestations of jSSc resemble adult SSc, but with better outcomes and survival. The etiology of jSSc appears to reflect adult-onset SSc, with similar inflammatory mediators and autoantibodies, but with a significant population of children with uncharacterized anti-nuclear antibodies. The genetics of patients with jSSc differ from women with SSc, resembling instead the genes of adult males with SSc, with additional HLA genes uniquely associated with childhood-onset disease. Current treatments are aimed at inhibiting the inflammatory aspect of disease, but important mechanisms of fibrosis regulated by dermal white adipose tissue dendritic cells may provide an avenue for targeting and potentially reversing the fibrotic stage.

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Section: Clinical Aspectsmentioning

confidence: 99%

Immunopathogenesis of Juvenile Systemic Sclerosis

Stevens

Torok

et al. 2019

Front. Immunol.

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“…Systemic sclerosis (SSc) is a rare, heterogeneous, chronic autoimmune disease that is characterized by fibrosis of the skin and is associated with high mortality [ 1 , 2 ]. The prevalence of SSc is estimated to be 7.2–33.9 and 13.5–44.3 per 100,000 individuals in Europe and North America, respectively [ 3 , 4 ]. Patients can develop organ involvement associated with SSc, including pulmonary hypertension, interstitial lung disease (ILD), gastrointestinal disorders, and cardiac and renal involvement [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

Healthcare Resource Utilization Among Patients in England with Systemic Sclerosis-Associated Interstitial Lung Disease: A Retrospective Database Analysis

et al. 2020

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Introduction Systemic sclerosis-associated interstitial lung disease (SSc-ILD) places a substantial burden on patients and healthcare systems. The objectives of this study were to describe clinical characteristics and assess healthcare resource utilization and costs of patients with SSc-ILD in England, compared with patients with non-pulmonary organ involvement related to SSc (SSc-OOI). Methods This population-based retrospective study used data from the Clinical Practice Research Datalink linked to Hospital Episode Statistics. Data were extracted from medical records dated January 1, 2005 to March 31, 2016. Patients with SSc were identified and placed in subgroups based on organ involvement: SSc-ILD, SSc-OOI, and both (SSc-ILD-OOI). Patients with SSc-ILD-OOI were included in both the SSc-ILD and SSc-OOI subgroups. All-cause healthcare costs, excluding medication costs, were calculated to 2016 British pounds sterling (£). Results This study included 675 patients with SSc: 174 (26%) had neither ILD nor other organ involvement (OOI); 127 (19%) had SSc-ILD; 477 (71%) had SSc-OOI; 103 (15%) had SSc-ILD-OOI. Age-weighted median [interquartile range (IQR)] annual healthcare costs per patient were: £1496 (£664–£2817) in SSc only; £6375 (£3451–£15,041) in SSc-ILD; £4084 (£1454–£10,105) in SSc-OOI; £6632 (£4023–£17,009) in SSc-ILD-OOI. In multivariate analysis, older age at diagnosis, diagnosis of anemia, and number of comorbid diseases were associated with higher yearly healthcare costs. Conclusion The annual healthcare cost for patients with SSc-ILD is substantial, and higher than that of patients with SSc-OOI or SSc only. These results quantify the economic burden of SSc-ILD in a real-world setting, and highlight the need for treatment of this disease. Electronic supplementary material The online version of this article (10.1007/s12325-020-01330-0) contains supplementary material, which is available to authorized users.

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“…The relatively small sample size may be considered as a limitation for this study: nevertheless, Systemic Sclerosis is a relatively rare disease, with approx. 20000 people living with the condition in the U.K. (Royle et al, 2018). Therefore, it was not possible to recruit more widely within our study period and with the limited funding that we had.…”

Section: Experimental Considerationsmentioning

confidence: 99%