Materiały źródłowe

• #1

  https://journals.lww.com/co-rheumatology/fulltext/2021/03000/scleroderma_epidemiology_update.4.aspx

  Systemic sclerosis (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs. This review aims to provide an overview and summary of the recent epidemiological studies in systemic sclerosis. […] Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring. Future research may emphasize enrichment of SSc patients in randomized trials who are more likely to progress or be treatment responsive, focused screening, and personalized patient care through the creation and validation of new SSc criteria and subsets.

• #2 Scleroderma epidemiology update

  https://ouci.dntb.gov.ua/en/works/9GGV1qK9/

  Scleroderma (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs. This review aims to provide an overview and summary of the recent epidemiological studies in systemic sclerosis. […] Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring. Future research may emphasize enrichment of SSc patients in randomized trials who are more likely to progress or be treatment responsive, focused screening, and personalized patient care through the creation and validation of new SSc criteria and subsets.

• #3 Scleroderma epidemiology update – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33481429/

  Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. […] However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring.

• #4 Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8516513/

  We aimed to conduct a systematic review and meta-analysis on the incidence and prevalence of SSc covering the entire literature. […] The overall pooled prevalence of SSc was 17.6 (95% CI 15.1, 20.5) per 100 000 and the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1, 1.9) per 100 000 person-years. […] The pooled incidence and prevalence in women were five times higher than in men. […] In this comprehensive review of the incidence and prevalence of SSc across the world, there was large heterogeneity among estimates, which should be taken into consideration when interpreting the results. […] Incidence and prevalence estimates of SSc vary considerably between studies. […] There is a temporal trend towards increased incidence and prevalence over calendar period. […] This increase is likely due to increased awareness and improvement in diagnostic methods and criteria.

• #5 Epidemiology of systemic sclerosis and systemic sclerosis-associated i | CLEP

  https://www.dovepress.com/epidemiology-of-systemic-sclerosis-and-systemic-sclerosis-associated-i-peer-reviewed-fulltext-article-CLEP

  Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease […] 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). […] 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.

• #6

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  In a recent meta-analysis, the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1-1.9) per 100,000 person-years and the overall pooled prevalence of SSc was 17.6 (95% CI 15.1-20.5) per 100,000 individuals, but the incidence rate ranged from 0.2 to 7.5 per 100,000 person-years, and the prevalence ranged from 3.1 to 144.5 per 100,000 individuals. This information highlights the great variability of data concerning the incidence and prevalence of SSc. There are several possible explanations for this variability. […] A first explanation of this observed variability could be the existence of significant differences in the design and methods in different studies, concerning both case identification (data sources and case ascertainment) and definition. In particular, although sometimes used also for prevalence and incidence evaluation, 1980 classification criteria suffered from poor sensitivity for early SSc and lcSSc, and were therefore not suitable to this task, inducing other groups to use also the classification criteria early SSc proposed by LeRoy and Medsger in 2001. Not surprisingly, the more recent ACR/EULAR 2013 criteria, which are more sensitive and useful also for early SSc patients classification, can identify more SSc cases, resulting in an around 40% higher estimate of SSc incidence and prevalence than the 1980 classification criteria.

• #7 Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8516513/

  We aimed to conduct a systematic review and meta-analysis on the incidence and prevalence of SSc covering the entire literature. […] The overall pooled prevalence of SSc was 17.6 (95% CI 15.1, 20.5) per 100 000 and the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1, 1.9) per 100 000 person-years. […] The pooled incidence and prevalence in women were five times higher than in men. […] In this comprehensive review of the incidence and prevalence of SSc across the world, there was large heterogeneity among estimates, which should be taken into consideration when interpreting the results. […] Incidence and prevalence estimates of SSc vary considerably between studies. […] There is a temporal trend towards increased incidence and prevalence over calendar period. […] This increase is likely due to increased awareness and improvement in diagnostic methods and criteria.

• #8 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The estimated incidence of systemic sclerosis in the United States is 20 cases per million population, and its prevalence has been estimated at 276 cases per million population, although the reported prevalence varies depending on the methodology used and the targeted population. […] An increased systemic sclerosis incidence and prevalence has been evident in the last 50 years. Although improved diagnosis and increased survival rate can partially account for this observation, it appears that there has been a real increase in its incidence. […] Systemic sclerosis occurs worldwide, although its reported prevalence varies significantly in different countries; for example, higher prevalence rates are reported in Europe and North and South America than in East Asia. […] Obtaining an exact estimate of prevalence is difficult because systemic sclerosis is frequently misdiagnosed.

• #9 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The estimated incidence of systemic sclerosis in the United States is 20 cases per million population, and its prevalence has been estimated at 276 cases per million population, although the reported prevalence varies depending on the methodology used and the targeted population. […] An increased systemic sclerosis incidence and prevalence has been evident in the last 50 years. Although improved diagnosis and increased survival rate can partially account for this observation, it appears that there has been a real increase in its incidence. […] Systemic sclerosis occurs worldwide, although its reported prevalence varies significantly in different countries; for example, higher prevalence rates are reported in Europe and North and South America than in East Asia. […] Obtaining an exact estimate of prevalence is difficult because systemic sclerosis is frequently misdiagnosed.

• #10 Who Gets Scleroderma? – National Scleroderma Foundation

  https://scleroderma.org/who-gets-scleroderma/

  Its estimated that about 300,000 Americans have scleroderma. About one third of those people have the systemic form of scleroderma. […] Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall, females with scleroderma outnumber males about 4-to-1. Other factors such as race and ethnic background, may determine the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. […] In African American individuals, the incidence of systemic sclerosis is higher than it is in non-African Americans. Among all African Americans, 23.7 people per million are diagnosed with systemic sclerosis, while the comparable rate among non-African Americans is 18.3 people per million diagnosed. […] The progression of the disease in African Americans can be different than in non-African Americans due to differences in autoantibodies. Generally speaking, African American are diagnosed at a younger age than non-African Americans are diagnosed. Additionally, African Americans far more frequently have the diffuse cutaneous form of systemic sclerosis and are more likely to have lung and other organ involvement than occurs among non-African Americans.

• #11 Prevalence and Incidence of Systemic Scleroderma in the US – Scleroderma Education Project

  https://sclerodermainfo.org/prevalence-and-incidence-of-systemic-scleroderma-in-the-us/

  It is very common to read in articles about systemic scleroderma that there are about 300,000 people in the US with this disease. This number is completely wrong, according to numerous research studies. […] The results were published in an article titled, Estimates of the Prevalence of Arthritis and Other Rheumatic Conditions in the United States in the January 2008 issue of Arthritis Rheumatism. That report estimated the number of US adults with diagnosed systemic sclerosis to be about 49,000. […] Another study on the prevalence (number of diagnosed cases) and incidence (number of new cases per year) of systemic scleroderma is the 2003 Mayes study. This study estimates that the prevalence of systemic sclerosis (the medical term for systemic scleroderma) in the US is about 240 per million adults and the incidence of new cases each year is about 20 per million. Based on 2013 US Census Bureau estimates of 242,470,820 adults in the US, this means that the number of diagnosed cases of systemic scleroderma is actually about 58,000 a long way from the 300,000 figure that is commonly used!

• #12

  https://journals.lww.com/co-rheumatology/fulltext/2021/03000/scleroderma_epidemiology_update.4.aspx

  Systemic sclerosis (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs. This review aims to provide an overview and summary of the recent epidemiological studies in systemic sclerosis. […] Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring. Future research may emphasize enrichment of SSc patients in randomized trials who are more likely to progress or be treatment responsive, focused screening, and personalized patient care through the creation and validation of new SSc criteria and subsets.

• #13 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The estimated incidence of systemic sclerosis in the United States is 20 cases per million population, and its prevalence has been estimated at 276 cases per million population, although the reported prevalence varies depending on the methodology used and the targeted population. […] An increased systemic sclerosis incidence and prevalence has been evident in the last 50 years. Although improved diagnosis and increased survival rate can partially account for this observation, it appears that there has been a real increase in its incidence. […] Systemic sclerosis occurs worldwide, although its reported prevalence varies significantly in different countries; for example, higher prevalence rates are reported in Europe and North and South America than in East Asia. […] Obtaining an exact estimate of prevalence is difficult because systemic sclerosis is frequently misdiagnosed.

• #14 Epidemiology of systemic sclerosis and systemic sclerosis-associated i | CLEP

  https://www.dovepress.com/epidemiology-of-systemic-sclerosis-and-systemic-sclerosis-associated-i-peer-reviewed-fulltext-article-CLEP

  Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease […] 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). […] 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.

• #15 Epidemiology of systemic sclerosis and systemic sclerosis-associated i | CLEP

  https://www.dovepress.com/epidemiology-of-systemic-sclerosis-and-systemic-sclerosis-associated-i-peer-reviewed-fulltext-article-CLEP

  Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease […] 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). […] 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.

• #16 Systemic Sclerosis (Scleroderma)

  https://patient.info/doctor/systemic-sclerosis-scleroderma

  SSc is present throughout the world and is represented in all ethnic groups. Incidence and prevalence figures vary widely and there appears to be a large geographical variation. […] Around the world, prevalence is reported to vary from 30-240 per million. In the UK, annual incidence is reported to be 3.7 per million and prevalence 31-88 per million. […] It seems to be more common in North America and Australia than in Europe. Women are affected more often than men, and those of African origin are also more affected. The peak age of onset is 40-50 years but it can affect any age group. It is rare in children.

• #17

  https://www.aaem.pl/Incidence-and-prevalence-of-Systemic-Sclerosis-SSc-in-Poland-differences-between-rural-and-urban-regions,72500,0,2.html

  Incidence and prevalence of Systemic Sclerosis (SSc) in Poland differences between rural and urban regions […] The purpose of this study was to present SSc epidemiology among hospitalized patients in Poland. […] Based on hospitalization registers, the average SSc incidence was estimated to be 1.9/100,000 per year and peak age of incidence was 55 years. The point prevalence was estimated to be 9.4/100,000 at the end of 2012. […] Analysis of hospitalization trends revealed that overall numbers of SSc hospitalizations increased, while first-time hospitalizations decreased between 2008-2012. Clusters of higher incidence were observed in more rural regions vs. urban regions. […] Estimated incidence of SSc in Poland was comparable to reported incidence in other European countries. Analysis of demographic factors and reports of clusters of higher incidence may suggest the existence of environmental risk factors for the development of SSc. These observations may require further research.

• #18 Scleroderma epidemiology update – PubMed

  https://pubmed.ncbi.nlm.nih.gov/33481429/

  Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. […] However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring.

• #19 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  However, it appears that there is higher frequency among Black individuals. […] Systemic sclerosis affects individuals of all races. […] However, incidence rates, severity and mortality may vary among ethnic groups. […] Studies have documented a higher general and age-specific incidence rate in Blacks than in Whites. […] It has also been shown that affected African Americans develop more severe disease and have poorer outcomes when compared with other ethnic groups. […] The highest prevalence of systemic sclerosis in the US is among the Oklahoma Choctaw Indians. […] The prevalence in the Choctaws is 469 cases per 100,000 population, which is higher than in nonfull-blood Choctaw (31 per 100,000) and 20 times higher than the national average. […] Similarly, a prevalence rate of 47 per 100,000 has been found in the indigenous peoples in Canada.

• #20 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  However, it appears that there is higher frequency among Black individuals. […] Systemic sclerosis affects individuals of all races. […] However, incidence rates, severity and mortality may vary among ethnic groups. […] Studies have documented a higher general and age-specific incidence rate in Blacks than in Whites. […] It has also been shown that affected African Americans develop more severe disease and have poorer outcomes when compared with other ethnic groups. […] The highest prevalence of systemic sclerosis in the US is among the Oklahoma Choctaw Indians. […] The prevalence in the Choctaws is 469 cases per 100,000 population, which is higher than in nonfull-blood Choctaw (31 per 100,000) and 20 times higher than the national average. […] Similarly, a prevalence rate of 47 per 100,000 has been found in the indigenous peoples in Canada.

• #21 Scleroderma epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Scleroderma_epidemiology_and_demographics

  Scleroderma commonly affects individuals between 20 to 50 years of age. […] Choctaw native Americans from southeastern Oklahoma were reported to have much higher prevalence of scleroderma than the general population. […] Scleroderma usually affects individuals of the African-American race more than the caucasian race. […] Females are more commonly affected by scleroderma than males. The female to male ratio is approximately ranging from 3:1 to 14:1. […] Scleroderma cases are more frequently reported in the United States than in continental Europe. […] Familial clustering of scleroderma has been reported in United States and Australia.

• #22 Scleroderma epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Scleroderma_epidemiology_and_demographics

  Scleroderma commonly affects individuals between 20 to 50 years of age. […] Choctaw native Americans from southeastern Oklahoma were reported to have much higher prevalence of scleroderma than the general population. […] Scleroderma usually affects individuals of the African-American race more than the caucasian race. […] Females are more commonly affected by scleroderma than males. The female to male ratio is approximately ranging from 3:1 to 14:1. […] Scleroderma cases are more frequently reported in the United States than in continental Europe. […] Familial clustering of scleroderma has been reported in United States and Australia.

• #23

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  Gender differences in SSc have multiple implications, including differences in epidemiology, pathogenesis, and clinical expression of disease. SSc is more common in females compared to males; the ratio between the genders varies in the literature between 3:1 and 14.5:1. However, it is more severe in males, which suffer more frequently from diffuse cutaneous and lung, heart, and kidney involvement. […] The mortality rate is still greater in SSc than in the general population. In a recent meta-analysis of 22 studies, the overall standardized mortality ratio (SMR) in SSc patients was 2.8 (95% CI 2.2-3.6). The SMR was very similar in European, North American, Asian, and Oceanian SSc populations. The SMR was numerically but not significantly higher in men than in women (3.5 versus 2.9), whereas it was greatly higher in the dcSSc than in the lcSSc subset (4.9 versus 2.0).

• #24 Incidence and prevalence of systemic sclerosis globally: a comprehensive systematic review and meta-analysis

  https://pmc.ncbi.nlm.nih.gov/articles/PMC8516513/

  We aimed to conduct a systematic review and meta-analysis on the incidence and prevalence of SSc covering the entire literature. […] The overall pooled prevalence of SSc was 17.6 (95% CI 15.1, 20.5) per 100 000 and the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1, 1.9) per 100 000 person-years. […] The pooled incidence and prevalence in women were five times higher than in men. […] In this comprehensive review of the incidence and prevalence of SSc across the world, there was large heterogeneity among estimates, which should be taken into consideration when interpreting the results. […] Incidence and prevalence estimates of SSc vary considerably between studies. […] There is a temporal trend towards increased incidence and prevalence over calendar period. […] This increase is likely due to increased awareness and improvement in diagnostic methods and criteria.

• #25 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The risk of systemic sclerosis is 4-9 times higher in women than in men. […] However, the mechanisms responsible for the disproportionately higher frequency in females have not been elucidated. […] A multicenter cohort of 2281 patients from the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry documented an overall female/male ratio of 8.2:1. […] Males were found to have a shorter time from onset of Raynaud phenomenon to systemic sclerosis diagnosis and an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers. […] Women demonstrated a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein. […] The peak onset occurs in individuals aged 30-50 years. […] Numerous cases occur in children, however, as well as in very old individuals.

• #26 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The risk of systemic sclerosis is 4-9 times higher in women than in men. […] However, the mechanisms responsible for the disproportionately higher frequency in females have not been elucidated. […] A multicenter cohort of 2281 patients from the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry documented an overall female/male ratio of 8.2:1. […] Males were found to have a shorter time from onset of Raynaud phenomenon to systemic sclerosis diagnosis and an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers. […] Women demonstrated a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein. […] The peak onset occurs in individuals aged 30-50 years. […] Numerous cases occur in children, however, as well as in very old individuals.

• #27 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The risk of systemic sclerosis is 4-9 times higher in women than in men. […] However, the mechanisms responsible for the disproportionately higher frequency in females have not been elucidated. […] A multicenter cohort of 2281 patients from the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry documented an overall female/male ratio of 8.2:1. […] Males were found to have a shorter time from onset of Raynaud phenomenon to systemic sclerosis diagnosis and an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers. […] Women demonstrated a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein. […] The peak onset occurs in individuals aged 30-50 years. […] Numerous cases occur in children, however, as well as in very old individuals.

• #28 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  However, it appears that there is higher frequency among Black individuals. […] Systemic sclerosis affects individuals of all races. […] However, incidence rates, severity and mortality may vary among ethnic groups. […] Studies have documented a higher general and age-specific incidence rate in Blacks than in Whites. […] It has also been shown that affected African Americans develop more severe disease and have poorer outcomes when compared with other ethnic groups. […] The highest prevalence of systemic sclerosis in the US is among the Oklahoma Choctaw Indians. […] The prevalence in the Choctaws is 469 cases per 100,000 population, which is higher than in nonfull-blood Choctaw (31 per 100,000) and 20 times higher than the national average. […] Similarly, a prevalence rate of 47 per 100,000 has been found in the indigenous peoples in Canada.

• #29 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  However, it appears that there is higher frequency among Black individuals. […] Systemic sclerosis affects individuals of all races. […] However, incidence rates, severity and mortality may vary among ethnic groups. […] Studies have documented a higher general and age-specific incidence rate in Blacks than in Whites. […] It has also been shown that affected African Americans develop more severe disease and have poorer outcomes when compared with other ethnic groups. […] The highest prevalence of systemic sclerosis in the US is among the Oklahoma Choctaw Indians. […] The prevalence in the Choctaws is 469 cases per 100,000 population, which is higher than in nonfull-blood Choctaw (31 per 100,000) and 20 times higher than the national average. […] Similarly, a prevalence rate of 47 per 100,000 has been found in the indigenous peoples in Canada.

• #30 Who Gets Scleroderma? – National Scleroderma Foundation

  https://scleroderma.org/who-gets-scleroderma/

  Its estimated that about 300,000 Americans have scleroderma. About one third of those people have the systemic form of scleroderma. […] Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall, females with scleroderma outnumber males about 4-to-1. Other factors such as race and ethnic background, may determine the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. […] In African American individuals, the incidence of systemic sclerosis is higher than it is in non-African Americans. Among all African Americans, 23.7 people per million are diagnosed with systemic sclerosis, while the comparable rate among non-African Americans is 18.3 people per million diagnosed. […] The progression of the disease in African Americans can be different than in non-African Americans due to differences in autoantibodies. Generally speaking, African American are diagnosed at a younger age than non-African Americans are diagnosed. Additionally, African Americans far more frequently have the diffuse cutaneous form of systemic sclerosis and are more likely to have lung and other organ involvement than occurs among non-African Americans.

• #31 Who Gets Scleroderma? – National Scleroderma Foundation

  https://scleroderma.org/who-gets-scleroderma/

  Its estimated that about 300,000 Americans have scleroderma. About one third of those people have the systemic form of scleroderma. […] Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall, females with scleroderma outnumber males about 4-to-1. Other factors such as race and ethnic background, may determine the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. […] In African American individuals, the incidence of systemic sclerosis is higher than it is in non-African Americans. Among all African Americans, 23.7 people per million are diagnosed with systemic sclerosis, while the comparable rate among non-African Americans is 18.3 people per million diagnosed. […] The progression of the disease in African Americans can be different than in non-African Americans due to differences in autoantibodies. Generally speaking, African American are diagnosed at a younger age than non-African Americans are diagnosed. Additionally, African Americans far more frequently have the diffuse cutaneous form of systemic sclerosis and are more likely to have lung and other organ involvement than occurs among non-African Americans.

• #32 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  However, it appears that there is higher frequency among Black individuals. […] Systemic sclerosis affects individuals of all races. […] However, incidence rates, severity and mortality may vary among ethnic groups. […] Studies have documented a higher general and age-specific incidence rate in Blacks than in Whites. […] It has also been shown that affected African Americans develop more severe disease and have poorer outcomes when compared with other ethnic groups. […] The highest prevalence of systemic sclerosis in the US is among the Oklahoma Choctaw Indians. […] The prevalence in the Choctaws is 469 cases per 100,000 population, which is higher than in nonfull-blood Choctaw (31 per 100,000) and 20 times higher than the national average. […] Similarly, a prevalence rate of 47 per 100,000 has been found in the indigenous peoples in Canada.

• #33 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The risk of systemic sclerosis is 4-9 times higher in women than in men. […] However, the mechanisms responsible for the disproportionately higher frequency in females have not been elucidated. […] A multicenter cohort of 2281 patients from the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry documented an overall female/male ratio of 8.2:1. […] Males were found to have a shorter time from onset of Raynaud phenomenon to systemic sclerosis diagnosis and an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers. […] Women demonstrated a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein. […] The peak onset occurs in individuals aged 30-50 years. […] Numerous cases occur in children, however, as well as in very old individuals.

• #34 Scleroderma epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Scleroderma_epidemiology_and_demographics

  Scleroderma commonly affects individuals between 20 to 50 years of age. […] Choctaw native Americans from southeastern Oklahoma were reported to have much higher prevalence of scleroderma than the general population. […] Scleroderma usually affects individuals of the African-American race more than the caucasian race. […] Females are more commonly affected by scleroderma than males. The female to male ratio is approximately ranging from 3:1 to 14:1. […] Scleroderma cases are more frequently reported in the United States than in continental Europe. […] Familial clustering of scleroderma has been reported in United States and Australia.

• #35 Scleroderma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/scleroderma?lang=us

  Scleroderma, also known as systemic sclerosis, is an autoimmune connective tissue disorder characterized by multisystem fibrosis and soft tissue calcification. […] Scleroderma is considered a disease of middle age (30-50 years of age). There is a strong female predilection (F:M 3:1), even more pronounced during reproductive years (F:M 10:1).

• #36 Who Gets Scleroderma? – National Scleroderma Foundation

  https://scleroderma.org/who-gets-scleroderma/

  Its estimated that about 300,000 Americans have scleroderma. About one third of those people have the systemic form of scleroderma. […] Localized scleroderma is more common in children, whereas systemic scleroderma is more common in adults. Overall, females with scleroderma outnumber males about 4-to-1. Other factors such as race and ethnic background, may determine the risk of getting scleroderma, the age of onset, and the pattern or severity of internal organ involvement. […] In African American individuals, the incidence of systemic sclerosis is higher than it is in non-African Americans. Among all African Americans, 23.7 people per million are diagnosed with systemic sclerosis, while the comparable rate among non-African Americans is 18.3 people per million diagnosed. […] The progression of the disease in African Americans can be different than in non-African Americans due to differences in autoantibodies. Generally speaking, African American are diagnosed at a younger age than non-African Americans are diagnosed. Additionally, African Americans far more frequently have the diffuse cutaneous form of systemic sclerosis and are more likely to have lung and other organ involvement than occurs among non-African Americans.

• #37

  https://www.aaem.pl/Incidence-and-prevalence-of-Systemic-Sclerosis-SSc-in-Poland-differences-between-rural-and-urban-regions,72500,0,2.html

  Incidence and prevalence of Systemic Sclerosis (SSc) in Poland differences between rural and urban regions […] The purpose of this study was to present SSc epidemiology among hospitalized patients in Poland. […] Based on hospitalization registers, the average SSc incidence was estimated to be 1.9/100,000 per year and peak age of incidence was 55 years. The point prevalence was estimated to be 9.4/100,000 at the end of 2012. […] Analysis of hospitalization trends revealed that overall numbers of SSc hospitalizations increased, while first-time hospitalizations decreased between 2008-2012. Clusters of higher incidence were observed in more rural regions vs. urban regions. […] Estimated incidence of SSc in Poland was comparable to reported incidence in other European countries. Analysis of demographic factors and reports of clusters of higher incidence may suggest the existence of environmental risk factors for the development of SSc. These observations may require further research.

• #38 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The risk of systemic sclerosis is 4-9 times higher in women than in men. […] However, the mechanisms responsible for the disproportionately higher frequency in females have not been elucidated. […] A multicenter cohort of 2281 patients from the Italian Systemic sclerosis PRogression INvestiGation (SPRING) registry documented an overall female/male ratio of 8.2:1. […] Males were found to have a shorter time from onset of Raynaud phenomenon to systemic sclerosis diagnosis and an increased prevalence of the diffuse cutaneous subset, renal crisis, and digital ulcers. […] Women demonstrated a significantly higher percentage of sicca syndrome, serum antinuclear antibodies, antiextractable nuclear antigens, anti-La/SSB, and anticentromere protein. […] The peak onset occurs in individuals aged 30-50 years. […] Numerous cases occur in children, however, as well as in very old individuals.

• #39 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  Systemic sclerosis (systemic scleroderma) is a connective tissue disease associated with autoimmunity, vasculopathy, and fibrosis. The annual incidence is estimated to be 10 to 20 cases per 1 million persons, whereas the prevalence is four to 253 cases per 1 million persons. Raynaud phenomenon and scleroderma (hardening of the skin) are the clinical hallmarks of the disease. Pulmonary fibrosis and pulmonary arterial hypertension are the leading causes of death. […] Patients who have systemic sclerosis can be classified into distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and patient survival. The most common subsets are limited cutaneous (approximately 60 percent of patients with systemic sclerosis) and diffuse cutaneous (approximately 35 percent of patients with systemic sclerosis).

• #40 Systemic Sclerosis Market (Scleroderma) Epidemiology Report to 2024

  https://www.prnewswire.com/in/news-releases/systemic-sclerosis-market-scleroderma-epidemiology-report-to-2024-565882271.html

  In the 7MM in 2014, the group age 50-59 years had the highest proportion of diagnosed prevalent cases of SSc at 25.51%. In 2014, the diagnosed prevalent cases of SSc in the 7MM were lower in men compared with women with 15.36% diagnosed prevalent cases in men and 84.64% diagnosed prevalent cases in women. Of all the diagnosed prevalent cases of SSc in the 7MM in 2014, more had experienced RP (126,151 cases) than a DU (67,099 cases). […] In each of the 7MM, approximately 95% of the diagnosed prevalent SSc cases had experienced RP. In all markets other than France, Germany, and Italy, 70.50% of the diagnosed prevalent cases of SSc were lSSc. In the 7MM in 2014, 18,395 of the diagnosed prevalent cases of dSSc had ILD, and 6,679 of the diagnosed prevalent cases of dSSc had kidney disease.

• #41 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  The estimated incidence of systemic sclerosis in the United States is 20 cases per million population, and its prevalence has been estimated at 276 cases per million population, although the reported prevalence varies depending on the methodology used and the targeted population. […] An increased systemic sclerosis incidence and prevalence has been evident in the last 50 years. Although improved diagnosis and increased survival rate can partially account for this observation, it appears that there has been a real increase in its incidence. […] Systemic sclerosis occurs worldwide, although its reported prevalence varies significantly in different countries; for example, higher prevalence rates are reported in Europe and North and South America than in East Asia. […] Obtaining an exact estimate of prevalence is difficult because systemic sclerosis is frequently misdiagnosed.

• #42

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  In a recent meta-analysis, the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1-1.9) per 100,000 person-years and the overall pooled prevalence of SSc was 17.6 (95% CI 15.1-20.5) per 100,000 individuals, but the incidence rate ranged from 0.2 to 7.5 per 100,000 person-years, and the prevalence ranged from 3.1 to 144.5 per 100,000 individuals. This information highlights the great variability of data concerning the incidence and prevalence of SSc. There are several possible explanations for this variability. […] A first explanation of this observed variability could be the existence of significant differences in the design and methods in different studies, concerning both case identification (data sources and case ascertainment) and definition. In particular, although sometimes used also for prevalence and incidence evaluation, 1980 classification criteria suffered from poor sensitivity for early SSc and lcSSc, and were therefore not suitable to this task, inducing other groups to use also the classification criteria early SSc proposed by LeRoy and Medsger in 2001. Not surprisingly, the more recent ACR/EULAR 2013 criteria, which are more sensitive and useful also for early SSc patients classification, can identify more SSc cases, resulting in an around 40% higher estimate of SSc incidence and prevalence than the 1980 classification criteria.

• #43 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  Systemic sclerosis renal crisis develops in 3 to 10 percent of all patients with systemic sclerosis and in 10 to 20 percent of those with rapidly progressive diffuse cutaneous systemic sclerosis; the greatest risk occurs within the first three years of the disease. […] Internal organ complications are common in patients with systemic sclerosis but are seldom symptomatic until the late stages of the disease; thus, routine screening for internal organ complications is essential.

• #44 Kidney disease in systemic sclerosis (scleroderma), including scleroderma renal crisis – UpToDate

  https://www.uptodate.com/contents/kidney-disease-in-systemic-sclerosis-scleroderma-including-scleroderma-renal-crisis

  SSc may affect only the skin and subjacent tissues but is generally associated with systemic involvement. […] The most serious kidney manifestation of SSc is scleroderma renal crisis (SRC), which occurs in a minority of patients. […] Autopsy studies reveal kidney pathology in approximately 60 percent of patients with SSc. […] In addition, as many as 50 percent of patients with SSc have markers of kidney disease, as manifested by mild proteinuria, elevated serum creatinine concentration, and/or hypertension. […] The most serious kidney manifestation of SSc is SRC. This life-threatening complication occurs in as many as 5 to 20 percent of patients with diffuse cutaneous SSc. […] SRC is a life-threatening complication of SSc. […] SRC is characterized by the following features: abrupt onset of moderate to marked hypertension, sometimes accompanied by manifestations of malignant hypertension such as hypertensive retinopathy and hypertensive encephalopathy.

• #45 Epidemiology and Survival of Systemic Sclerosis–Sarcoidosis Overlap Syndrome | The Journal of Rheumatology

  https://www.jrheum.org/content/50/5/656

  We evaluated the epidemiology, manifestations, serology, comorbidities, and survival among patients with systemic sclerosis (SSc) with and without sarcoidosis. […] We included 1977 patients (1971 with SSc, 6 with SScsarcoidosis) with a SScsarcoidosis prevalence of 0.30%. […] Sarcoidosis in SSc is rare but appears to occur more frequently than in the general population. […] We found that although SScsarcoidosis is rare, sarcoidosis among patients with SSc is twice as common as in the general population. […] We found no statistically significant differences in either short-term or long-term survival between patients with SSc and SScsarcoidosis. […] It could be that both diseases are being better managed. […] Patients with SScsarcoidosis should be monitored carefully as it is associated with an increased risk of stroke.

• #46 Epidemiology, Clinical Characteristics of Systemic Sclerosis vs SSc-SLE Overlap Syndrome – Rheumatology Advisor

  https://www.rheumatologyadvisor.com/news/epidemiology-clinical-characteristics-of-systemic-sclerosis-vs-ssc-sle-overlap-syndrome/

  Patients with SSc-SLE overlap syndrome were younger at diagnosis, had higher rates of pulmonary arterial hypertension, and less frequently had SSc cutaneous manifestations. […] The prevalence of SSc-SLE overlap syndrome ranges from 8.4% to 14.7%, but there is relatively little information in the literature about its clinical or epidemiologic characteristics. […] The prevalence of SSc-SLE was 6.8%. […] Study strengths included a large sample size, extended follow-up, and thorough mortality data. […] They recommended that all patients with SSc-SLE be carefully monitored for development of digital ulcers, interstitial lung disease, and renal crisis as part of their routine management.

• #47

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  Gender differences in SSc have multiple implications, including differences in epidemiology, pathogenesis, and clinical expression of disease. SSc is more common in females compared to males; the ratio between the genders varies in the literature between 3:1 and 14.5:1. However, it is more severe in males, which suffer more frequently from diffuse cutaneous and lung, heart, and kidney involvement. […] The mortality rate is still greater in SSc than in the general population. In a recent meta-analysis of 22 studies, the overall standardized mortality ratio (SMR) in SSc patients was 2.8 (95% CI 2.2-3.6). The SMR was very similar in European, North American, Asian, and Oceanian SSc populations. The SMR was numerically but not significantly higher in men than in women (3.5 versus 2.9), whereas it was greatly higher in the dcSSc than in the lcSSc subset (4.9 versus 2.0).

• #48

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  Gender differences in SSc have multiple implications, including differences in epidemiology, pathogenesis, and clinical expression of disease. SSc is more common in females compared to males; the ratio between the genders varies in the literature between 3:1 and 14.5:1. However, it is more severe in males, which suffer more frequently from diffuse cutaneous and lung, heart, and kidney involvement. […] The mortality rate is still greater in SSc than in the general population. In a recent meta-analysis of 22 studies, the overall standardized mortality ratio (SMR) in SSc patients was 2.8 (95% CI 2.2-3.6). The SMR was very similar in European, North American, Asian, and Oceanian SSc populations. The SMR was numerically but not significantly higher in men than in women (3.5 versus 2.9), whereas it was greatly higher in the dcSSc than in the lcSSc subset (4.9 versus 2.0).

• #49 Epidemiology of systemic sclerosis and systemic sclerosis-associated i | CLEP

  https://www.dovepress.com/epidemiology-of-systemic-sclerosis-and-systemic-sclerosis-associated-i-peer-reviewed-fulltext-article-CLEP

  Epidemiology of systemic sclerosis and systemic sclerosis-associated interstitial lung disease […] 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). […] 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.

• #50 Systemic Sclerosis/Scleroderma: A Treatable Multisystem Disease | AAFP

  https://www.aafp.org/pubs/afp/issues/2008/1015/p961.html

  Systemic sclerosis (systemic scleroderma) is a connective tissue disease associated with autoimmunity, vasculopathy, and fibrosis. The annual incidence is estimated to be 10 to 20 cases per 1 million persons, whereas the prevalence is four to 253 cases per 1 million persons. Raynaud phenomenon and scleroderma (hardening of the skin) are the clinical hallmarks of the disease. Pulmonary fibrosis and pulmonary arterial hypertension are the leading causes of death. […] Patients who have systemic sclerosis can be classified into distinct clinical subsets with different patterns of skin and internal organ involvement, autoantibody production, and patient survival. The most common subsets are limited cutaneous (approximately 60 percent of patients with systemic sclerosis) and diffuse cutaneous (approximately 35 percent of patients with systemic sclerosis).

• #51 Digital ulcers in systemic sclerosis: their epidemiology, clinical characteristics, and associated clinical and economic burden | Arthritis Research & Therapy | Full Text

  https://arthritis-research.biomedcentral.com/articles/10.1186/s13075-019-2080-y

  Despite DU being associated with morbidity and reduced HRQoL, our study indicates that their presence alone does not reduce survival. […] To reduce the clinical burden of DUs, additional research is needed to determine effective interventions and management plans such as the development of a DU specific multidisciplinary clinic.

• #52 Epidemiology and Survival of Systemic Sclerosis–Sarcoidosis Overlap Syndrome | The Journal of Rheumatology

  https://www.jrheum.org/content/50/5/656

  We evaluated the epidemiology, manifestations, serology, comorbidities, and survival among patients with systemic sclerosis (SSc) with and without sarcoidosis. […] We included 1977 patients (1971 with SSc, 6 with SScsarcoidosis) with a SScsarcoidosis prevalence of 0.30%. […] Sarcoidosis in SSc is rare but appears to occur more frequently than in the general population. […] We found that although SScsarcoidosis is rare, sarcoidosis among patients with SSc is twice as common as in the general population. […] We found no statistically significant differences in either short-term or long-term survival between patients with SSc and SScsarcoidosis. […] It could be that both diseases are being better managed. […] Patients with SScsarcoidosis should be monitored carefully as it is associated with an increased risk of stroke.

• #53

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  In a recent meta-analysis, the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1-1.9) per 100,000 person-years and the overall pooled prevalence of SSc was 17.6 (95% CI 15.1-20.5) per 100,000 individuals, but the incidence rate ranged from 0.2 to 7.5 per 100,000 person-years, and the prevalence ranged from 3.1 to 144.5 per 100,000 individuals. This information highlights the great variability of data concerning the incidence and prevalence of SSc. There are several possible explanations for this variability. […] A first explanation of this observed variability could be the existence of significant differences in the design and methods in different studies, concerning both case identification (data sources and case ascertainment) and definition. In particular, although sometimes used also for prevalence and incidence evaluation, 1980 classification criteria suffered from poor sensitivity for early SSc and lcSSc, and were therefore not suitable to this task, inducing other groups to use also the classification criteria early SSc proposed by LeRoy and Medsger in 2001. Not surprisingly, the more recent ACR/EULAR 2013 criteria, which are more sensitive and useful also for early SSc patients classification, can identify more SSc cases, resulting in an around 40% higher estimate of SSc incidence and prevalence than the 1980 classification criteria.

• #54

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  A second explanation is the timeframe in which the epidemiological studies were conducted may be another source of variability. Results from a meta-regression of prevalence against calendar period indicated that more recent studies reported higher incidence and prevalence estimate. […] A third reason for the variability in the observed incidence and prevalence could be related to geographical differences in SSc distribution that might also contribute to the variation of demographic parameters. Information on this issue from some large areas of the world (e.g., Africa and South America) is scanty. The disease is more common in North America or Australia than in East Asia. […] The highest prevalence of SSc was reported in Choctaw Native American from Oklahoma (66 cases per 100,000 using the 1980 criteria over the 1990-1994 interval). In Alberta, Canada, the highest prevalence of SSc was also observed in the indigenous people, in which numerous diverse Tribal Nations were represented.

• #55

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  In a recent meta-analysis, the overall pooled incidence rate of SSc was 1.4 (95% CI 1.1-1.9) per 100,000 person-years and the overall pooled prevalence of SSc was 17.6 (95% CI 15.1-20.5) per 100,000 individuals, but the incidence rate ranged from 0.2 to 7.5 per 100,000 person-years, and the prevalence ranged from 3.1 to 144.5 per 100,000 individuals. This information highlights the great variability of data concerning the incidence and prevalence of SSc. There are several possible explanations for this variability. […] A first explanation of this observed variability could be the existence of significant differences in the design and methods in different studies, concerning both case identification (data sources and case ascertainment) and definition. In particular, although sometimes used also for prevalence and incidence evaluation, 1980 classification criteria suffered from poor sensitivity for early SSc and lcSSc, and were therefore not suitable to this task, inducing other groups to use also the classification criteria early SSc proposed by LeRoy and Medsger in 2001. Not surprisingly, the more recent ACR/EULAR 2013 criteria, which are more sensitive and useful also for early SSc patients classification, can identify more SSc cases, resulting in an around 40% higher estimate of SSc incidence and prevalence than the 1980 classification criteria.

• #56

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  A second explanation is the timeframe in which the epidemiological studies were conducted may be another source of variability. Results from a meta-regression of prevalence against calendar period indicated that more recent studies reported higher incidence and prevalence estimate. […] A third reason for the variability in the observed incidence and prevalence could be related to geographical differences in SSc distribution that might also contribute to the variation of demographic parameters. Information on this issue from some large areas of the world (e.g., Africa and South America) is scanty. The disease is more common in North America or Australia than in East Asia. […] The highest prevalence of SSc was reported in Choctaw Native American from Oklahoma (66 cases per 100,000 using the 1980 criteria over the 1990-1994 interval). In Alberta, Canada, the highest prevalence of SSc was also observed in the indigenous people, in which numerous diverse Tribal Nations were represented.

• #57

  https://link.springer.com/article/10.1007/s12016-022-08929-x

  A second explanation is the timeframe in which the epidemiological studies were conducted may be another source of variability. Results from a meta-regression of prevalence against calendar period indicated that more recent studies reported higher incidence and prevalence estimate. […] A third reason for the variability in the observed incidence and prevalence could be related to geographical differences in SSc distribution that might also contribute to the variation of demographic parameters. Information on this issue from some large areas of the world (e.g., Africa and South America) is scanty. The disease is more common in North America or Australia than in East Asia. […] The highest prevalence of SSc was reported in Choctaw Native American from Oklahoma (66 cases per 100,000 using the 1980 criteria over the 1990-1994 interval). In Alberta, Canada, the highest prevalence of SSc was also observed in the indigenous people, in which numerous diverse Tribal Nations were represented.

• #58 Scleroderma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/331864-overview

  However, it appears that there is higher frequency among Black individuals. […] Systemic sclerosis affects individuals of all races. […] However, incidence rates, severity and mortality may vary among ethnic groups. […] Studies have documented a higher general and age-specific incidence rate in Blacks than in Whites. […] It has also been shown that affected African Americans develop more severe disease and have poorer outcomes when compared with other ethnic groups. […] The highest prevalence of systemic sclerosis in the US is among the Oklahoma Choctaw Indians. […] The prevalence in the Choctaws is 469 cases per 100,000 population, which is higher than in nonfull-blood Choctaw (31 per 100,000) and 20 times higher than the national average. […] Similarly, a prevalence rate of 47 per 100,000 has been found in the indigenous peoples in Canada.

• #59

  https://clarivate.com/life-sciences-healthcare/report/epidim0059-biopharma-scleroderma-systemic-sclerosis-epidemiology-mature-markets/

  Clarivate Epidemiologys coverage of scleroderma (systemic sclerosis) comprises epidemiological estimates of key patient populations across 14 mature and emerging pharmaceutical markets worldwide. […] We report both the incidence and prevalence of scleroderma (systemic sclerosis) for each country, as well as annualized case counts projected to the national population. […] Most patient populations are forecast over a period of 20 years for the major mature pharmaceutical markets and 10 years for the other countries covered in this report. […] Clarivate Epidemiologys scleroderma (systemic sclerosis) forecast will answer the following questions: Of all people diagnosed with scleroderma (systemic sclerosis), how many in each country across the major mature pharmaceutical markets are drug-treated?

• #60

  https://clarivate.com/life-sciences-healthcare/report/epidim0059-biopharma-scleroderma-systemic-sclerosis-epidemiology-mature-markets/

  How will demographic trends, such as population aging and improving life expectancy, affect the epidemiology of scleroderma (systemic sclerosis) over the forecast period? […] In total, Clarivate Epidemiology forecasts eleven scleroderma (systemic sclerosis) patient populations, as follows: Diagnosed incident cases, Diagnosed prevalent cases, Diagnosed prevalent cases of diffuse cutaneous SSc, Diagnosed prevalent cases of limited cutaneous SSc, Diagnosed prevalent cases of limited or unspecified SSc, Diagnosed prevalent cases with comorbid ILD, Diagnosed prevalent cases with no comorbid ILD, Diagnosed prevalent cases with comorbid PAH, Diagnosed prevalent cases with no comorbid PAH, Drug-treated prevalent cases, Non-drug-treated prevalent cases.

• #61

  https://clarivate.com/life-sciences-healthcare/report/epidim0059-biopharma-scleroderma-systemic-sclerosis-epidemiology-mature-markets/

  Clarivate Epidemiologys coverage of scleroderma (systemic sclerosis) comprises epidemiological estimates of key patient populations across 14 mature and emerging pharmaceutical markets worldwide. […] We report both the incidence and prevalence of scleroderma (systemic sclerosis) for each country, as well as annualized case counts projected to the national population. […] Most patient populations are forecast over a period of 20 years for the major mature pharmaceutical markets and 10 years for the other countries covered in this report. […] Clarivate Epidemiologys scleroderma (systemic sclerosis) forecast will answer the following questions: Of all people diagnosed with scleroderma (systemic sclerosis), how many in each country across the major mature pharmaceutical markets are drug-treated?

• #62 Systemic Sclerosis (Scleroderma) – Epidemiology Forecast to 2030

  https://www.globaldata.com/store/report/systemic-sclerosis-scleroderma-epidemiology-analysis/

  Systemic sclerosis (SSc) is an autoimmune disorder of small arteries, microvessels, and connective tissue. […] This report provides an overview of the risk factors, comorbidities, and the global and historical epidemiological trends for SSc in the seven major markets (7MM: US, France, Germany, Italy, Spain, UK, and Japan). […] In the 7MM, GlobalData epidemiologists forecast an increase in the diagnosed prevalent cases of SSc from 170,921 cases in 2020 to 187,518 cases in 2030, at an AGR of 0.97% over the forecast period. […] The report includes a 10-year epidemiological forecast for the diagnosed prevalent cases of SSc segmented by sex and age (ages 18 years). […] The SSc epidemiology report and model were written and developed by Masters- and PhD-level epidemiologists. […] The Epidemiology Report is in-depth, high quality, transparent and market-driven, providing expert analysis of disease trends in the 7MM. […] The Epidemiology Model is easy to navigate, interactive with dashboards, and epidemiology-based with transparent and consistent methodologies.

• #63 Systemic Scleroderma – Epidemiology Forecast to 2032

  https://www.researchandmarkets.com/reports/5525669/systemic-scleroderma-epidemiology-forecast-to?srsltid=AfmBOopqbNLJ1Odb-v5mfvF3yp04U9hitPa7ehE8OdGp_m_DM8DDtbR0

  This „Systemic Scleroderma – Epidemiology Forecast to 2032” report delivers an in-depth understanding of the disease, historical and forecasted Systemic Scleroderma epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan. […] The Systemic Scleroderma epidemiology report gives a thorough understanding of the Systemic Scleroderma by including details such as disease definition, symptoms, causes, pathophysiology, and diagnosis. It also provides treatment algorithms and treatment guidelines for Systemic Scleroderma in the US, Europe, and Japan. The report covers the detailed information of the Systemic Scleroderma epidemiology scenario in seven major countries (US, EU5, and Japan). […] The Systemic Scleroderma epidemiology division provides insights about historical and current patient pool and forecasted trend for every seven major countries. The Systemic Scleroderma epidemiology data are studied through all possible division to give a better understanding of the Disease scenario in 7MM. The Systemic Scleroderma epidemiology segment covers the epidemiology data in the US, EU5 countries (Germany, Spain, Italy, France, and the UK), and Japan from 2019 to 2032. It also helps recognize the causes of current and forecasted trends by exploring numerous studies, survey reports and views of key opinion leaders.

• #64 Localized Scleroderma – Epidemiology Forecast to 2032

  https://www.researchandmarkets.com/reports/5525998/localized-scleroderma-epidemiology-forecast-to?srsltid=AfmBOoo1b2k8eSyHbwuJaTWqn-o9pZw-egdBQWFNWy104wCFnD1kGVL_

  The „Localized Scleroderma – Epidemiology Forecast to 2032” report delivers an in-depth understanding of the disease, historical and forecasted Localized Scleroderma epidemiology in the 7MM, i.e., the United States, EU5 (Germany, Spain, Italy, France, and the United Kingdom), and Japan. […] The Localized Scleroderma epidemiology report gives a thorough understanding of the Localized Scleroderma by including details such as disease definition, symptoms, causes, pathophysiology, and diagnosis. […] The Localized Scleroderma epidemiology division provides insights about historical and current patient pool and forecasted trend for every seven major countries. […] The Localized Scleroderma epidemiology segment covers the epidemiology data in the US, EU5 countries (Germany, Spain, Italy, France, and the UK), and Japan from 2019 to 2032.

• #65 Scleroderma epidemiology update

  https://ouci.dntb.gov.ua/en/works/9GGV1qK9/

  Scleroderma (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs. This review aims to provide an overview and summary of the recent epidemiological studies in systemic sclerosis. […] Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring. Future research may emphasize enrichment of SSc patients in randomized trials who are more likely to progress or be treatment responsive, focused screening, and personalized patient care through the creation and validation of new SSc criteria and subsets.

• #66 Scleroderma epidemiology update

  https://ouci.dntb.gov.ua/en/works/9GGV1qK9/

  Scleroderma (scleroderma, SSc) is a rare multisystem autoimmune disease characterized by autoantibodies, vasculopathy, and fibrosis of the skin and internal organs. This review aims to provide an overview and summary of the recent epidemiological studies in systemic sclerosis. […] Global trends of scleroderma demonstrate greater prevalence of SSc in European, North, and South American patients compared with East Asian patients. However, the greatest prevalence (47 in 100 000), was found among the indigenous peoples in Canada. […] Understanding the epidemiological factors in SSc enables patient care through patient classification, prognostication, and monitoring. Future research may emphasize enrichment of SSc patients in randomized trials who are more likely to progress or be treatment responsive, focused screening, and personalized patient care through the creation and validation of new SSc criteria and subsets.

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