Trends in systemic sclerosis and systemic sclerosis-related pulmonary arterial hypertension mortality in the USA

Ratanawatkul, Pailin; Solomon, Joshua J.; Kim, Darlene; George, M Patricia; McGibbon, Lia R. Matarrese; Demoruelle, M. Kristen; Maleki-Fischbach, Mehrnaz; Amigues, Isabelle; Kastsianok, Liudmila; Pérez, Evans R. Fernández

doi:10.1183/23120541.00309-2019

Cited by 7 publications

(7 citation statements)

References 26 publications

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“…However, the ratio rose slightly among males aged 65–74 years, from 71.3% to 71.8%. Ratanawatkul et al [ 23 ] indicated a similar pattern using UCD and MCD data. Among patients with SSc, the risk of dying from cancer, cardiopulmonary complications, infections, or malignancies increases with age; consequently, SSc is less likely to be selected as the UCD in such cases.…”

Section: Discussionmentioning

confidence: 62%

“…Individuals with SSc-related PAH (SSc-PAH) have a mortality risk that is threefold higher than the individuals with the idiopathic PAH [ 22 ]. In the United States, SSc- PAH mortality stayed steady from 2003 to 2008, then declined by 3% annually from 2008 to 2016 [ 23 ]. Furthermore, a meta-analysis revealed that SSc-PAH was significantly associated with a greater pooled risk of mortality than SSc without PAH (RR 3.12, 95% Cl 2.44–3.98) [ 24 ].…”

Section: Discussionmentioning

confidence: 99%

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Secular Trends in Systemic Sclerosis Mortality in the United States from 1981 to 2020

2022

IJERPH

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Background: Systemic sclerosis (SSc) has the highest mortality rate among autoimmune disorders. Individuals with SSc frequently die from complications or infections related to SSc. Nonetheless, the sex–age–period interaction of SSc is complex and remains unclear. The study aims to analyze the secular trend of SSc mortality based on data regarding underlying cause of death (UCD) and multiple causes of death (MCD) and clarify the sex–age interaction with time. Methods: The multiple-cause mortality statistics provided by the National Center for Health Statistics were used to identify all deaths in the United States from 1981 to 2020 in which SSc was indicated anywhere on the death certificates. The age-standardized mortality rate (ASMR) was determined for both sexes, as well as the variations in these rates. Joinpoint regression analysis was utilized to determine the annual percentage change (APC) of ASMR. Results: A total of 44,672 and 66,259 individuals who died between 1981 and 2020 were identified based on the UCD and MCD data, respectively. According to the UCD data, SSc-related AMSR (SSc-ASMR) of the male and female decedents, respectively, declined from 5.01 and 1.94 in 1981–1990 to 4.77 and 1.32 in 2011–2020, respectively (mortality rate ratio 0.95, 95% confidence interval 0.92–0.98). From 1986 to 1999, the APC of SSc-ASMR in female decedents decreased except for those aged 45–64 years (APC 2.1%, p = 0.002). For MCD analysis, in trend 1, only APC of SSc-ASMR in male decedents aged 45–64 years decreased. The SSc-ASMR of both male and female decedents fell on trend 2 arm. In 2011–2020, the ratio of UCD to MCD increased across all age groups for both sexes compared to 1981–1990. Overall, compared to the male decedents, the SSc-ASMR in female decedents increased significantly before 1999, peaked in 1999, followed by continuous decrease until 2020 according to UCD and MCD statistics. Conclusions: Over the past four decades, the SSc deaths based on the MCD data were 1.48 times more than the UCD data, and the proportion of UCD over MCD increased over time. The SSc-ASMRs in all the sex–age groups significantly decreased over the past two decades. Notably, the mortality rate ratio of women to men with SSc increased in the past four decades.

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Section: Discussionmentioning

confidence: 62%

Section: Discussionmentioning

confidence: 99%

Secular Trends in Systemic Sclerosis Mortality in the United States from 1981 to 2020

2022

IJERPH

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“…Our findings complement a recent study that also reported that the age-adjusted mortality rate of scleroderma-ILD remained stable from 2003 to 2016 in the United States (average annual percentage change, 1.2, 95% CI: À0.2 to 2.6). 5 Similarly, ILD was not a predominant contributing cause of death in scleroderma (1,010 of 25,175 records; 4%). Studies that examine mortality rates in US decedents with DPM-ILD are not available for comparison.…”

Section: Discussionmentioning

confidence: 95%

Connective Tissue Disease-Interstitial Lung Disease-Associated Mortality Rates and Years of Potential Life Lost in the United States

Salinas

Solomon

Pérez

2021

Chest

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Connective tissue diseases (CTD) are a diverse group of systemic disorders characterized by autoimmunemediated organ damage. Interstitial lung disease (ILD) is an extra-articular complication that is seen commonly in many CTDs and, if left untreated, can lead to significant morbidity and death. 1,2 Few studies have focused on the epidemiologic factors of CTD-ILD, and none have investigated the impact of premature death on a nationwide scale. We sought to determine the prevalence and contemporary mortality trends of CTD-ILD in the United States and to quantify the annual loss from these deaths by computing years of potential life lost to age 65 (YPLL 65 ), considered as a loss of years from a traditional working life, and the number of years of potential life lost before the average life expectancy (YPLL 75 and YPLL 85 ). MethodsWe analyzed the multiple-cause-of-death records from the Centers for Disease Control and Prevention's Wide-ranging Online Data for Epidemiologic Research Database from 1999 to 2017. 3 This online program compiles data on death certificates for all US decedents. Each multiple-cause-of-death record contains a single underlying cause of death and up to 20 additional causes of death, along with demographic data. CTD-ILD-associated death was defined as decedents $18 years of age whose multiple-cause-of-death record included rheumatoid arthritis (code M05.0 to M06.9), scleroderma (code M34), or dermatomyositis and polymyositis (DPM; code M33) and ILD (code, J84) coded according to the International Classification of Diseases, 10th Revision. These three CTDs were included because they are among the most common ones associated with ILD.

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“…Nonetheless, due to the general lack of data, in particular wide population-based studies in the literature, the comparison of our results with previous studies, is only possible for specific diseases. For instance, referring to the drop of 46% in the risk of death observed in ‘diseases of the musculoskeletal system and connective tissue’ between the 2000–2009 and 2010–2018 periods, an analysis on systemic sclerosis, the most important disorder of autoimmune rheumatological disease, observed a reduction in the mortality rate from 2003 to 2016 in the United States [ 29 ], possibly due to the improved early diagnosis and consequently treatment, which decrease the impact of complications and comorbidities [ 28 ].…”

Section: Discussionmentioning

confidence: 99%

Survival of patients with rare diseases: a population-based study in Tuscany (Italy)

Gorini

Coi

Mezzasalma

et al. 2021

Orphanet J Rare Dis

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Background Rare diseases (RDs) encompass a heterogeneous group of life-threatening or chronically debilitating conditions that individually affect a small number of subjects but overall represent a major public health issue globally. There are still limited data on RD burden due to the paucity of large population-based epidemiological studies. The aim of this research was to provide survival estimates of patients with a RD residing in Tuscany, Italy. Methods Cases collected in the Rare Diseases Registry of Tuscany with diagnosis between 1st January 2000 and 31th December 2018 were linked to the regional health databases in order to retrieve information on mortality of all subjects. Survival at 1, 5 and 10 years from diagnosis with 95% confidence intervals (CI) was estimated by sex, age class, nosological group and subgroup using the Kaplan–Meier method. The effect of sex, age and period of diagnosis (years 2000–2009 or 2010–2018) on survival was estimated using Cox proportional hazards regression. Results Survival at 1, 5 and 10 years from diagnosis was 97.3%, 88.8% and 80.8%, respectively. Respiratory diseases and peripheral and central nervous system disorders were characterized by the lowest survival at 5 and 10 years. Despite a modest higher prevalence of RDs among females (54.0% of the total), male cases had a significant increased risk of death (hazard ratio, HR 1.48, 95% CI 1.38–1.58). Cases diagnosed during 2010–2018 period had a risk of death significantly lower than those diagnosed during 2000–2009 (HR 0.81, 95% CI 0.82–0.96), especially for immune system disorders (HR 0.48, 95% CI 0.26–0.87), circulatory system diseases (HR 0.61, 95% CI 0.45–0.84) and diseases of the musculoskeletal system and connective tissue (HR 0.64, 95% CI 0.49–0.84). Conclusions An earlier diagnosis as well as the improvement in the efficacy of treatment resulted in a decreased risk of death over the years for specific RDs. The linkage between a population-based registry and other regional databases exploited in this study provides a large and accurate mass of data capable of estimating patients’ life-expectancy and increasing knowledge on the collective burden of RDs.

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Trends in systemic sclerosis and systemic sclerosis-related pulmonary arterial hypertension mortality in the USA

Cited by 7 publications

References 26 publications

Secular Trends in Systemic Sclerosis Mortality in the United States from 1981 to 2020

Secular Trends in Systemic Sclerosis Mortality in the United States from 1981 to 2020

Connective Tissue Disease-Interstitial Lung Disease-Associated Mortality Rates and Years of Potential Life Lost in the United States

Survival of patients with rare diseases: a population-based study in Tuscany (Italy)

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