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Original Research

Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease

, , &
Pages 257-273 | Published online: 18 Apr 2019
 

Abstract

Background: Interstitial lung disease (ILD) is one of the leading causes of mortality in patients with systemic sclerosis (SSc). To further understand this patient population, we present the first systematic review on the epidemiology of SSc and SSc-associated ILD (SSc-ILD).

Methods: Bibliographic databases and web sources were searched for studies including patients with SSc and SSc-ILD in Europe and North America (United States and Canada). The systematic review was limited to publications in English, German, French, Spanish, Italian, and Portuguese, published between January 1, 2000 and February 29, 2016. For all publications included in the review, the methodologic quality was assessed. For each dimension and region, data availability in terms of quantity and consistency of reported findings was evaluated.

Results: Fifty publications reporting epidemiologic data (prevalence, incidence, demographic profile, and survival and mortality) were included; 39 included patients with SSc and 16 included patients with SSc-ILD. The reported prevalence of SSc was 7.2–33.9 and 13.5–44.3 per 100,000 individuals in Europe and North America, respectively. Annual incidence estimates were 0.6–2.3 and 1.4–5.6 per 100,000 individuals in Europe and North America, respectively. Associated ILD was present in ~35% of the patients in Europe and ~52% of the patients in North America. In Europe, a study estimated the prevalence and annual incidence of SSc-ILD at 1.7–4.2 and 0.1–0.4 per 100,000 individuals, respectively. In both Europe and North America, SSc-ILD was diagnosed at a slightly older age than SSc, with both presentations of the disease affecting 2–3 times more women than men. Ten-year survival in patients with SSc was reported at 65–73% in Europe and 54–82% in North America, with cardiorespiratory manifestations (including ILD) associated with poor prognosis.

Conclusion: This systematic review confirms that SSc and SSc-ILD are rare, with geographic variation in prevalence and incidence.

Acknowledgments

The authors received no compensation related to the development of the manuscript. Medical writing assistance, in the form of the preparation and revision of the manuscript, was supported financially by Boehringer Ingelheim International GmbH and provided by Lianne Young, BSc (Hons), ISMPP CMPP™, of Complete HealthVizion, and Ben Daniels, BSc (Hons), BA, of AMICULUM Limited under the authors’ conceptual direction and based on feedback from the authors. Patrice Verpillat’s current affiliation is Merck KGaA, Darmstadt, Germany.

Abbreviations list

ACR, American College of Rheumatology; CI, confidence interval; dcSSc, diffuse cutaneous systemic sclerosis; EULAR, European League Against Rheumatism; EUSTAR, EULAR Scleroderma Trials and Research; HRCT, high-resolution computed tomography; ILD, interstitial lung disease; NOS, Newcastle–Ottawa scale; OR, odds ratio; PHAROS, Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma; POEMAS, Pulmonary Hypertension Screening, a Multidisciplinary Approach in Scleroderma; PRISMA, Preferred Reporting Items for Systematic reviews and Meta-Analyses checklist; SD, standard deviation; SRC, scleroderma renal crisis; SSc, systemic sclerosis; SSc-ILD, systemic sclerosis-associated interstitial lung disease; THIN, The Health Improvement Network.

Author contributions

The authors meet the criteria for authorship as recommended by the International Committee of Medical Journal Editors. All authors contributed to data analysis, drafting or revising the article, gave final approval of the version to be published, and agree to be accountable for all aspects of the work.  AB and PV were involved in the planning and conduct of the systematic review, interpretation of data, and development of the manuscript. NH and LW were involved in the interpretation of data and development of the manuscript.

Disclosure

AB is an employee of YolaRx Consultants, which received funding from Boehringer Ingelheim for the conduct of this systematic literature review. NH and LW are employees of Boehringer Ingelheim. PV is a former employee of Boehringer Ingelheim. The authors report no further conflicts of interest in this work.