Epidemiology and trends in survival of systemic sclerosis in Olmsted County (1980–2018): A population-based study

2021 ◽  
pp. 239719832110268
Author(s):  
Caitrin M Coffey ◽  
Yasser A Radwan ◽  
Avneek S Sandhu ◽  
Cynthia S Crowson ◽  
Philippe R Bauer ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
General Population ◽  
Confidence Interval ◽  
Population Based ◽  
Classification Criteria ◽  
Standardized Mortality Ratio ◽  
Olmsted County ◽  
Incident Cases ◽  
Over Time ◽  
Age And Sex

Background/Purpose: To update the epidemiology of systemic sclerosis and evaluate the performance of the American College of Rheumatology/European League Against Rheumatism 2013 versus 1980 American Rheumatism Association classification criteria in a US population-based cohort. Methods: An inception cohort of patients with incident systemic sclerosis from 1 January 1980 through 31 December 2018, in Olmsted County, Minnesota, was identified based on comprehensive individual medical record review. Incidence and prevalence rates were age- and sex-adjusted to the 2010 US white population. Survival rates were compared with expected rates in the general population. Fulfillment of 1980 and 2013 classification criteria was ascertained. Results: A total of 85 incident cases of systemic sclerosis (91% female, mean age = 55.4 ± 16 years) and 49 prevalent cases on 1 January 2015 were identified. The overall age- and sex-adjusted annual incidence was 25 (95% confidence interval = 20–31) per million population, with no change in incidence over time. The age- and sex-adjusted prevalence was 436 (95% confidence interval = 313–558) per 1,000,000 population. 77 (91%) patients fulfilled the 2013 classification criteria, and 38 (45%) fulfilled the 1980 criteria. Mortality among patients with systemic sclerosis was significantly higher in comparison to the general population, with a standardized mortality ratio of 2.48 (95% confidence interval = 1.76–3.39) and no evidence of improvement over time. Conclusion: Systemic sclerosis developed in 25 persons/million/year with no change over the 39-year study period. The 2013 classification criteria perform significantly better than the 1980 criteria but failed to classify 9% of patients. Systemic sclerosis portends a 2.5-fold higher risk of mortality than the general population, with no evidence of improved survival over time.

scholarly journals POS0838 EPIDEMIOLOGY AND TRENDS IN SURVIVAL OF SYSTEMIC SCLEROSIS IN OLMSTED COUNTY: A POPULATION-BASED STUDY (1980-2018)

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 673.1-673
Author(s):  
C. Coffey ◽  
Y. Radwan ◽  
A. Sandhu ◽  
C. S. Crowson ◽  
P. Bauer ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
General Population ◽  
Survival Rates ◽  
Population Based ◽  
Classification Criteria ◽  
Olmsted County ◽  
Digital Ischemia ◽  
Incident Cases ◽  
Over Time ◽  
Age And Sex

Background:Systemic sclerosis (SSc) is a complex immune-mediated disease with heterogeneous manifestations, which is characterized by vasculopathy and fibrosis of the skin and visceral organs. Mortality associated with SSc exceeds that of other rheumatic diseases, though population-based studies assessing recent trends in survival are lacking.Objectives:We aimed to determine the incidence and prevalence of physician-diagnosed SSc in a population-based cohort over a 39-year time period, and assess for trends in survival over time.Methods:Medical records of patients with a diagnosis or suspicion of SSc in a geographically well-defined area from Jan 1, 1980 to Dec 31, 2018 were reviewed to identify incident cases of SSc. Cases were defined by physician diagnosis of SSc, and fulfillment of the 2013 ACR/EULAR classification criteria was ascertained. Prevalent cases of SSc on Jan 1, 2015 were also identified. Incidence and prevalence rates were age- and sex-adjusted to the 2010 U.S. white population. Survival rates were compared with expected rates in the general population.Results:85 incident cases of SSc (91% female, mean age 55.4 ± 16 y) and 49 prevalent cases on Jan 1, 2015 were identified. Patients had a mean 11.7 (SD 9.4) years of follow-up available. The overall age and sex adjusted annual incidence for 1980-2018 was 2.5 (95% CI: 2.0-3.1) per 100,000 population, with no change in incidence over time (p=0.32). The age-adjusted incidence was 4.4 (95% CI: 3.4-5.4) for females, and 0.56 (95% CI: 0.16-0.96) for males per 100,000 population. The age- and sex-adjusted prevalence on Jan 1, 2015 was 43.6 (95% CI: 31.3-55.8) per 100,000 population.77 (91%) patients fulfilled the 2013 classification criteria; 38 (45%) fulfilled 1980 criteria. 70 (82%) had limited cutaneous involvement, 12 (14%) had diffuse cutaneous involvement, and 3 (4%) had sine scleroderma. At SSc diagnosis, 80 (94%) patients had Raynaud’s, 43 (51%) had sclerodactyly, 39 (46%) had telangiectasias, 14/48 (29%) had abnormal nailfold capillaries, 16/35 (46%) had digital ulcers or fingertip scarring, 8 (9%) had interstitial lung disease (ILD), and 7 (8%) had pulmonary arterial hypertension (PAH). 77/82 patients (91%) had a positive antinuclear antibody. 44 (52%) had a known SSc-related autoantibody: 32 (73%) with anti-centromere, 9 (20%) with anti-Scl-70, and 4 (9%) with anti-RNA-polymerase III.Survival was 77% (95% CI: 69-87) at 5 years, 66% (95% CI: 56-78) at 10 years, and 42% (95% CI: 30-57) at 20 years, with no evidence of improved survival over time (p=0.46). Age (Hazard ratio [HR]: 1.49 per 10 year increase; 95% CI 1.19-1.88), smoking at time of diagnosis (HR: 2.37; 95% CI: 1.05-5.34), digital ischemia (HR: 2.54; 95% CI: 1.33-4.87), ILD (HR: 4.00; 95% CI: 2.11-7.59), and PAH (HR: 4.30; 95% CI: 2.24-8.25) had significant associations with mortality. Survival of patients with SSc was poorer than the general population (standardized mortality ratio: 2.48; 95% CI: 1.76-3.39).Conclusion:The average incidence of SSc in this population-based cohort spanning 39 years was 2.5 per 100,000 population, with no change in incidence over time. Age, smoking, digital ischemia, ILD and PAH were risk factors for poorer survival. Overall survival for patients with SSc is worse than that of the general population and shows no improvement over time, suggesting continued need for improved diagnostic and treatment measures.Figure 1.Survival of 85 Olmsted County residents with SSc compared with expected survival rates from Minnesota lifetables (observed: solid line, expected: dashed line).Disclosure of Interests:None declared

scholarly journals Hospitalization Rates are Highest in the First 5 Years of Systemic Sclerosis: Results from a Population-based Cohort (1980-2016)

2020 ◽  
pp. jrheum.200737
Author(s):  
Caitrin M. Coffey ◽  
Avneek S. Sandhu ◽  
Cynthia S. Crowson ◽  
Sara J. Achenbach ◽  
Eric L. Matteson ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Healthcare Resource ◽  
Geographic Area ◽  
Population Based ◽  
Care Needs ◽  
Readmission Rates ◽  
Lengths Of Stay ◽  
Hospitalization Rates ◽  
Incident Cases ◽  
Age And Sex

Objective Few studies have estimated the healthcare resource usage of patients with systemic sclerosis (SSc). The purpose of this study was to compare hospitalization among incident cases of SSc vs age- and sex-matched comparators. Methods A retrospective, population-based cohort of patients with SSc in Olmsted County, MN from Jan 1, 1980 to Dec 31, 2016 was assembled. A 2:1 cohort of age- and sex-matched patients without SSc from the same population was randomly selected for comparison. All hospitalizations in the geographic area from Jan. 1, 1987 to Sept. 30, 2018 were obtained. Rates of hospitalization, lengths of stay, and readmissions were compared between groups. Results 76 incident SSc cases and 155 non-SSc comparators (mean age of 56 ± 16 years at diagnosis/index, 91% female) were included. Rates of hospitalization among cases and comparators were 31.9 and 17.9 per 100 person-years, respectively (rate ratio [RR]:1.78; 95% confidence interval (CI):1.52-2.08). Hospitalization rates were higher in patients with SSc than comparators during the first 5 years after SSc diagnosis (RR: 2.16; 95%CI: 1.70-2.74). This difference decreased over time and was no longer significant at ≥15 years after SSc incidence/index. Lengths of stay (median (IQR) 4 (2-6) vs 3(2-6); p=0.52) and readmission rates (25% vs 23%; p=0.51) were similar between groups. Conclusion Patients with SSc were hospitalized more frequently than comparators, indicating high inpatient care needs in this population. Hospitalization rates were highest during the first 5 years following SSc diagnosis.

Systemic sclerosis portends increased risk of conduction and rhythm abnormalities at diagnosis and during disease course: A US population-based cohort

2021 ◽  
pp. 239719832110340
Author(s):  
Yasser A Radwan ◽  
Reto D Kurmann ◽  
Avneek S Sandhu ◽  
Edward A El-Am ◽  
Cynthia S Crowson ◽  
...  
Keyword(s):  
Risk Factors ◽  
Pulmonary Hypertension ◽  
Systemic Sclerosis ◽  
Confidence Interval ◽  
Cumulative Incidence ◽  
Disease Onset ◽  
Population Based ◽  
Hazard Ratio ◽  
Conduction Disorders ◽  
Increased Risk

Objectives: To study the incidence, risk factors, and outcomes of conduction and rhythm disorders in a population-based cohort of patients with systemic sclerosis versus nonsystemic sclerosis comparators. Methods: An incident cohort of patients with systemic sclerosis (1980–2016) from Olmsted County, MN, was compared to age- and sex-matched nonsystemic sclerosis subjects (1:2). Electrocardiograms, Holter electrocardiograms, and a need for cardiac interventions were reviewed to determine the occurrence of any conduction or rhythm abnormalities. Results: Seventy-eight incident systemic sclerosis cases and 156 comparators were identified (mean age 56 years, 91% female). The prevalence of any conduction disorder before systemic sclerosis diagnosis compared to nonsystemic sclerosis subjects was 15% versus 7% ( p = 0.06), and any rhythm disorder was 18% versus 13% ( p = 0.33). During a median follow-up of 10.5 years in patients with systemic sclerosis and 13.0 years in nonsystemic sclerosis comparators, conduction disorders developed in 25 patients with systemic sclerosis with cumulative incidence of 20.5% (95% confidence interval: 12.4%–34.1%) versus 28 nonsystemic sclerosis patients with cumulative incidence of 10.4% (95% confidence interval: 6.2%–17.4%) (hazard ratio: 2.57; 95% confidence interval: 1.48–4.45), while rhythm disorders developed in 27 patients with systemic sclerosis with cumulative incidence of 27.3% (95% confidence interval: 17.9%–41.6%) versus 43 nonsystemic sclerosis patients with cumulative incidence of 18.0% (95% confidence interval: 12.3%–26.4%) (hazard ratio: 1.62; 95% confidence interval: 1.00–2.64). Age, pulmonary hypertension, and smoking were identified as risk factors. Conclusion: Patients with systemic sclerosis have an increased risk of conduction and rhythm disorders both at disease onset and over time, compared to nonsystemic sclerosis patients. These findings warrant increased vigilance and screening for electrocardiogram abnormalities in systemic sclerosis patients with pulmonary hypertension.

scholarly journals Patterns of Survival Among Patients With Myeloproliferative Neoplasms Diagnosed in Sweden From 1973 to 2008: A Population-Based Study

2012 ◽  
Vol 30 (24) ◽  
pp. 2995-3001 ◽  
Author(s):  
Malin Hultcrantz ◽  
Sigurdur Yngvi Kristinsson ◽  
Therese M.-L. Andersson ◽  
Ola Landgren ◽  
Sandra Eloranta ◽  
...  
Keyword(s):  
General Population ◽  
Life Expectancy ◽  
Excess Mortality ◽  
Myeloproliferative Neoplasms ◽  
Treatment Options ◽  
Large Population ◽  
Relative Survival ◽  
Population Based ◽  
Population Based Study ◽  
Over Time

PurposeReported survival in patients with myeloproliferative neoplasms (MPNs) shows great variation. Patients with primary myelofibrosis (PMF) have substantially reduced life expectancy, whereas patients with polycythemia vera (PV) and essential thrombocythemia (ET) have moderately reduced survival in most, but not all, studies. We conducted a large population-based study to establish patterns of survival in more than 9,000 patients with MPNs.Patients and MethodsWe identified 9,384 patients with MPNs (from the Swedish Cancer Register) diagnosed from 1973 to 2008 (divided into four calendar periods) with follow-up to 2009. Relative survival ratios (RSRs) and excess mortality rate ratios were computed as measures of survival.ResultsPatient survival was considerably lower in all MPN subtypes compared with expected survival in the general population, reflected in 10-year RSRs of 0.64 (95% CI, 0.62 to 0.67) in patients with PV, 0.68 (95% CI, 0.64 to 0.71) in those with ET, and 0.21 (95% CI, 0.18 to 0.25) in those with PMF. Excess mortality was observed in patients with any MPN subtype during all four calendar periods (P < .001). Survival improved significantly over time (P < .001); however, the improvement was less pronounced after the year 2000 and was confined to patients with PV and ET.ConclusionWe found patients with any MPN subtype to have significantly reduced life expectancy compared with the general population. The improvement over time is most likely explained by better overall clinical management of patients with MPN. The decreased life expectancy even in the most recent calendar period emphasizes the need for new treatment options for these patients.

Abstract P372: Coronary Angiography Use, Results and Revascularization Trends in Olmsted County, Minnesota, 2000 to 2015

Circulation ◽  
2018 ◽  
Vol 137 (suppl_1) ◽  
Author(s):  
Yariv L Gerber ◽  
Susan Weston ◽  
Jill Killian ◽  
Matteo Fabbri ◽  
Sheila Manemann ◽  
...  
Keyword(s):  
Coronary Angiography ◽  
Regression Models ◽  
Direct Method ◽  
Coronary Revascularization ◽  
Temporal Trends ◽  
Olmsted County ◽  
Left Main ◽  
Logistic Regression Models ◽  
Over Time ◽  
Age And Sex

Background: A decline in all forms of coronary revascularization has been previously reported. Whether the decline has continued after the turn of the century is unknown as well as whether it is influenced by trends in the use and results of coronary angiography. Methods: All diagnostic and therapeutic coronary procedures performed among Olmsted County, MN residents from 2000-2015 were analyzed. Standardized rates (per 100,000 population) were calculated applying the direct method and temporal trends compared using Poisson regression models. Trends in disease severity, defined as 3-vessel and/or left main coronary artery disease (CAD), were assessed using logistic regression models. Results: Between 2000 and 2015, 11,691 coronary angiographies were performed (63% men; 54% ≥ 65 years of age). The age- and sex-standardized rates of angiography decreased over time (p trend < 0.001; Figure). Overall, 30% of the subjects had 3-vessel and/or left main CAD, and this proportion decreased over time (age- and sex-adjusted odds ratios (95% CI) for severe CAD: 0.68 (0.62-0.76) in 2005-2009 and 0.69 (0.63-0.77) in 2010-2015 compared with 2000-2004). Among 5,222 coronary revascularization procedures performed, 78% were PCI and 22% CABG. The age- and sex-standardized rates of any revascularization declined during the study period, reflecting temporal decreases in both PCI and CABG (all p trend < 0.001; Figure). The declines in angiography rates, CAD severity, and revascularization utilization were consistently greater in women than men (all P for interactions < 0.01). Conclusions: Declines in all forms of coronary revascularization, which were greater in women than men, have occurred in Olmsted County, MN, from 2000-2015. The declines occurred in the context of fewer angiograms performed in the population and reduced severity of anatomic CAD.

Risk of bleeding events among patients with systemic sclerosis and the general population in the UK: a large population-based cohort study

2019 ◽  
Vol 39 (1) ◽  
pp. 19-26
Author(s):  
Alexander Michel ◽  
Antonio González-Pérez ◽  
María E. Sáez ◽  
Luis A. García Rodríguez
Keyword(s):  
Cohort Study ◽  
Systemic Sclerosis ◽  
General Population ◽  
Large Population ◽  
Population Based ◽  
Bleeding Events ◽  
Risk Of Bleeding ◽  
The Uk

scholarly journals Death From Stroke in End-Stage Kidney Disease

Stroke ◽  
2019 ◽  
Vol 50 (2) ◽  
pp. 487-490 ◽  
Author(s):  
Nicole L. De La Mata ◽  
Philip Masson ◽  
Rustam Al-Shahi Salman ◽  
Patrick J. Kelly ◽  
Angela C. Webster
Keyword(s):  
Kidney Disease ◽  
General Population ◽  
Standardized Mortality Ratio ◽  
Stroke Mortality ◽  
End Stage Kidney Disease ◽  
Mortality Ratio ◽  
Standardized Mortality Ratios ◽  
Using Data ◽  
End Stage ◽  
Over Time

Background and Purpose— People with end-stage kidney disease (ESKD) are at greater risk of stroke. We aimed to compare stroke mortality between the ESKD population and the general population. Methods— We included all patients with incident ESKD in Australia, 1980 to 2013, and New Zealand, 1988 to 2012. The primary cause of death was ascertained using data linkage with national death registers. We produced standardized mortality ratios for stroke deaths, by age, sex, and calendar year. Results— We included 60 823 patients with ESKD, where 941 stroke deaths occurred during 381 874 person-years. Patients with ESKD had >3× the stroke deaths compared with the general population (standardized mortality ratio, 3.4; 95% CI, 3.2–3.6), markedly higher in younger people and women. The greatest excess was in intracerebral hemorrhages (standardized mortality ratio, 5.2; 95% CI, 4.5–5.9). Excess stroke deaths in patients with ESKD decreased over time, although were still double in 2013 (2013 standardized mortality ratio, 2.1; 95% CI, 1.5–2.9). Conclusions— People with ESKD experience much greater stroke mortality with the greatest difference for women and younger people. However, mortality has improved over time.

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