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

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

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

Journal of Scleroderma and Related Disorders ◽

10.1177/23971983211026853 ◽

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.

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

The Journal of Rheumatology ◽

10.3899/jrheum.200737 ◽

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.

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AB0541 IMPROVED SURVIVAL IN PATIENTS WITH GIANT CELL ARTERITIS: A POPULATION-BASED COHORT STUDY

Annals of the Rheumatic Diseases ◽

10.1136/annrheumdis-2020-eular.230 ◽

2020 ◽

Vol 79 (Suppl 1) ◽

pp. 1567.1-1567

Author(s):

T. Garvey ◽

C. S. Crowson ◽

M. Koster ◽

E. Matteson ◽

K. J. Warrington

Keyword(s):

General Population ◽

Giant Cell Arteritis ◽

Giant Cell ◽

Survival Rates ◽

Mortality Rates ◽

Population Based ◽

Hazard Ratio ◽

Olmsted County ◽

Research Support ◽

Specific Mortality

Background:In previous studies patients with giant cell arteritis (GCA) have had survival rates that are similar to the general population.Objectives:To investigate survival trends and cause-specific mortality in patients diagnosed with GCA over a 60-year period.Methods:We assembled a population-based incidence cohort of patients with GCA diagnosed between 1950 and 2009. All patients were included if they met the American College of Rheumatology (ACR) 1990 Criteria for the Classification of GCA. Patients diagnosed between 2000 and 2009 could also be included if they met the following criteria: age greater than or equal to 50 years, elevated inflammatory markers, and radiographic evidence of large-vessel vasculitis attributed to GCA. A non-GCA comparison cohort was assembled from the same underlying population for each patient with GCA. Patients were followed until death, last contact, or December 31st, 2018. Survival trends were analyzed by grouping patients into the following categories according to year of GCA diagnosis: Group A 1950-1979; Group B 1980-1989; Group C 1990-1999; and Group D 2000-2009. Mortality rates were estimated using the Kaplan-Meier method and were compared with expected mortality rates for persons of the same age, sex, and calendar year, as estimated by regional population life tables. Cause-specific mortality was obtained from death certificates for patients in both cohorts. The causes were grouped according to ICD-9 chapters and hazard ratios were estimated against the non-GCA comparators.Results:The study population included 245 incident cases of GCA: 194 (79%) women and 51 (21%) men with mean age (±SD) of 76.2 (±8.3) years and median follow-up of 10.6 years. There was no overall difference in survival between the GCA cohort and the general population. The 2-, 5-, and 10-year survival rates (95% CI) were 89% (86, 93), 76% (70, 81), and 56% (50, 63) respectively with a standardized mortality ratio of 0.99 (0.86, 1.14). The standardized mortality ratios for Groups A, B, C, and D were 0.83 (0.57, 1.17), 0.92 (0.63, 1.3), 1.21 (0.85, 1.69), 0.70 (0.50, 0.95), respectively. The overall all-cause mortality adjusted for age, sex, and calendar-year was similar between the GCA patients and their comparators with a hazard ratio of 1.03 (0.84, 1.24). Mortality due to neoplasms was significantly lower in the GCA cohort with a hazard ratio of 0.53 (0.3, 0.92). Other cause-specific mortalities were not significantly different between the groups.Conclusion:In this population-based cohort of patients with GCA diagnosed over a 60-year period, the survival of patients diagnosed in recent years was significantly better than that of the general population. The explanation for this novel finding is unclear, but likely to be multifactorial. In this study the number of deaths due to neoplasm in the GCA group was significantly lower.References:[1]Chandran AK, et al. Incidence of Giant Cell Arteritis in Olmsted County, Minnesota, over a 60-year period 1950-2009. Scand J Rheumatol. 2015;44(3):215-218.[2]Mohammad A. et al. Rate of comorbidities in Giant Cell Arteritis: A Population-based Study. J Rheumatol. 2017;44(1):84-90.[3]Salvarani C. et al. Reappraisal of the epidemiology of giant cell arteritis in Olmsted County, Minnesota, over a fifty-year period. Arthritis Rheum. 2004;51(2):264-8.Acknowledgments:This study was made possible using the resources of the Rochester Epidemiology Project, which is supported by the National Institute on Aging of the National Institutes of Health (NIH) under Award Number R01 AG034676, and CTSA Grant Number UL1 TR000135 from the National Center for Advancing Translational Sciences (NCATS), a component of the NIH. The content is solely the responsibility of the authors and does not necessarily represent the official views of the NIH.Disclosure of Interests:Thomas Garvey: None declared, Cynthia S. Crowson Grant/research support from: Pfizer research grant, Matthew Koster: None declared, Eric Matteson Grant/research support from: Pfizer, Consultant of: Boehringer Ingelheim, Gilead, TympoBio, Arena Pharmaceuticals, Speakers bureau: Simply Speaking, Kenneth J Warrington: None declared

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Outpatient healthcare utilization among incident cases of systemic sclerosis: results from a population-based US cohort (1988–2016)

Scandinavian Journal of Rheumatology ◽

10.1080/03009742.2021.1932581 ◽

2021 ◽

pp. 1-6

Author(s):

CM Coffey ◽

AS Sandhu ◽

CS Crowson ◽

D Asante ◽

EL Matteson ◽

...

Keyword(s):

Systemic Sclerosis ◽

Healthcare Utilization ◽

Population Based ◽

Incident Cases

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

Journal of Clinical Oncology ◽

10.1200/jco.2012.42.1925 ◽

2012 ◽

Vol 30 (24) ◽

pp. 2995-3001 ◽

Cited By ~ 112

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.

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EPID-10. GLIOMA INCIDENCE AND SURVIVAL BY INCOME LEVEL IN THE UNITED STATES GENERAL POPULATION AGES AND ABOVE FROM 2000-2018

Neuro-Oncology ◽

10.1093/neuonc/noab196.343 ◽

2021 ◽

Vol 23 (Supplement_6) ◽

pp. vi87-vi88

Author(s):

Jennifer Murillo ◽

Elizabeth Anyanda ◽

Jason Huang

Keyword(s):

United States ◽

General Population ◽

Survival Data ◽

Survival Rates ◽

The United States ◽

Income Level ◽

Population Based ◽

Research Database ◽

Income Levels ◽

Average Survival

Abstract Gliomas are the most common primary malignant brain tumor in the United States with previous studies showing the incidence varied by age, sex, and race or ethnicity. Survival after diagnosis has also been shown to vary by these factors. Also, socioeconomic status and its association with various cancers have also been studied at length over time. PURPOSE: The purpose of our research was to quantify the differences in incidence and survival rates of gliomas in 15 years and older by income level. METHODS: This population-based study obtained incidence and survival data from the Incidence-SEER Research Database the general population. Average age incidence were generated by glioma groups and grouped by income levels. Survival rates were generated by overall glioma diagnosis grouped by observed survival at 12, 24, 36, 48 and 60 months and by again by income levels. The analysis included 94,207 patients with glioma diagnosed in those aged 15 years or older. RESULTS: Overall, 94, 207 patients diagnosed with glioma were analyzed. Of these, 1,089 (1.16%) fell into the < $35k group, 1,684 (1.79%) in the $35k-$40k group, 3,473 (3.69%) in the $40k-$45k group, 5,647 (5.99%) in the $45k-$50k group, 7,138 (7.58%) in the $50k-$55k group, 6,468 (6.87%) in the $55k-$60k group, 15,348 (16.29%) in the $60k-$65k group, 13,216 (14.03%) in the $65k-$70k group, 9,035 (9.59%) in the $70k-$75k group, and 31,109 (33.02%) fell in > $75k group. The data was also broken further down into survivability showing average survival. CONCLUSION: Incidence of glioma and 12, 24, 36, 48 and 60 month survival rates after diagnosis vary significantly by income level with higher income level greater than $75,000+ having higher incidence and higher survival rates compared with lower income levels. Further research is needed to help determine risk factors and barriers to care to help reveal health disparities.

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

Circulation ◽

10.1161/circ.137.suppl_1.p372 ◽

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.

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