<p>Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease</p>

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.

Short-Term Lung Function Changes and Predictors of Progressive Systemic Sclerosis–Related Interstitial Lung Disease

“…Our results for the incidence of ILD according to subtypes were similar. Our study had similar results to European, North American, and other studies done in Thailand that found that SSc is found mostly in females 8,10,11 . Pulmonary involvement was related to specific ethic, socioeconomic, and behavioral factors in SSc 15 .…”

“…The pathogenesis of SSc-ILD remains unclear; however, three steps of pathogenesis have been proposed: (1) persistent and repeated injuries of endothelial cells; (2) activation of innate/adaptive immunity; and (3) fibroblast recruitment/activation 4 . From previous studies, the associated factors of lung function decline in SSc-ILD are black males, diffuse type, and presence of antinuclear antibodies, anti-topoisomerase I, and anti-Th/To, but the absence of anti-centromere antibodies [4][5][6][7][8][9][10][11][12] .…”

Lung function is associated with minimal EQ-5D changes over time in patients with systemic sclerosis

“…Interstitial lung disease (ILD) is frequent in systemic sclerosis (SSc), with a prevalence reported between 35 and 52% [1]. Considering that ILD represents a major cause of mortality in SSc [2], preventing the progression of respiratory impairment is of utmost importance to improve life expectancy of SSc patients and it has been the target of several investigational studies.…”

Ongoing challenges in pulmonary fibrosis and insights from the nintedanib clinical programme

“…ILD is one of the leading causes of morbidity and mortality in SSc [9,104,137]. SSc-ILD has an estimated prevalence of 1.7-4.2 per 100,000 individuals in Europe [138]. The SENSCIS trial investigated the use of nintedanib in patients with SSc-ILD.…”