Crowson, Cynthia S.

ObjectiveWe aimed to determine the population‐based incidence, prevalence, and mortality of dermatomyositis (DM) using European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) criteria.MethodsThis population‐based cohort study included incident DM from January 1, 1995 to December 31, 2019. We manually reviewed all individuals with at least 1 code for DM or polymyositis to determine if they met EULAR/ACR criteria, subspecialty physician diagnosis, and/or Bohan and Peter criteria. We age‐ and sex‐adjusted incidence and prevalence estimates to the US non‐Hispanic White year 2000 population and estimated prevalence on January 1, 2015. Standardized mortality ratios (SMRs) with 95% confidence intervals (95% CIs) compared observed to expected mortality adjusting for age, sex, and year.ResultsWe identified 40 cases of verified DM, with 29 cases incident in Olmsted County from 1995 to 2019. The mean age was 57 years, 26 (90%) were female, and 12 (41%) had clinically amyopathic DM (CADM). The median follow‐up time was 8.2 years. The overall adjusted incidence of DM was 1.1 (95% CI 0.7–1.5) per 100,000 person‐years, and prevalence was 13 (95% CI 6–19) per 100,000. The SMR was significantly elevated among the myopathic DM cases (3.1 [95% CI 1.1–6.8]) but not CADM cases (1.1 [95% CI 0.2–3.3]). The positive predictive value of ≥2 DM codes was only 40 of 82 (49%).ConclusionThis population‐based study found that DM incidence and prevalence were higher than previously reported. Mortality was significantly elevated for myopathic DM but not for CADM.

ObjectiveTreat‐to‐target (T2T) and shared decision‐making are valued features of current guidelines for rheumatoid arthritis (RA) management. Although T2T has demonstrated value for improving RA outcomes, implementation remains inconsistent and lacks standardization and procedures for including patient input. We sought to better understand the impact of shared decisions on T2T and how treatment goal discussions between patients and providers impact RA treatment improvement and satisfaction.MethodsAn anonymous, web‐based questionnaire was presented to United States residents aged 18 years or older with a self‐reported diagnosis of RA by a medical professional with 28 questions regarding socio‐demographics, RA disease activity (DA), diagnosis, treatments, outcomes, and goals. Analyses included descriptive statistics with χ2 and rank sum tests for comparisons.ResultsThe questionnaire was completed by 907 people (mean age of 58 years; mean 11 years since diagnosis; 90% female). The majority (571; 63%) did not discuss RA treatment goals with providers. Patients engaging in treatment goal discussions with their providers were three times more likely to be satisfied with their treatment plans. Patients discussing treatment goals with their providers were more likely to have improved DA levels and 68% more likely to reach remission.ConclusionA majority of patients with RA report having no treatment goal discussion with their providers; however, these discussions are associated with greater DA improvement and treatment satisfaction. Further research should seek understanding of how shared treatment goal discussions relate to successful RA management and explore the development of practical tools to implement them in regular clinic practice as part of a T2T regimen.

Abstract Many neuro-oncology studies commonly assess the association between a prognostic factor (predictor) and disease or outcome, such as the association between age and glioma. Predictors can be continuous (eg, age) or categorical (eg, race/ethnicity). Effects of categorical predictors are frequently easier to visualize and interpret than effects of continuous variables. This makes it an attractive, and seemingly justifiable, option to subdivide the continuous predictors into categories (eg, age <50 years vs age ≥50 years). However, this approach results in loss of information (and power) compared to the continuous version. This review outlines the use cases for continuous and categorized predictors and provides tips and pitfalls for interpretation of these approaches.

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.

Abstract Aims To assess differences in estimated cardiovascular disease (CVD) risk among rheumatoid arthritis (RA) patients from different world regions and to evaluate the management and goal attainment of lipids and blood pressure (BP). Methods and results The survey of CVD risk factors in patients with RA was conducted in 14 503 patients from 19 countries during 2014–19. The treatment goal for BP was <140/90 mmHg. CVD risk prediction and lipid goals were according to the 2016 European guidelines. Overall, 21% had a very high estimated risk of CVD, ranging from 5% in Mexico, 15% in Asia, 19% in Northern Europe, to 31% in Central and Eastern Europe and 30% in North America. Of the 52% with indication for lipid-lowering treatment (LLT), 44% were using LLT. The lipid goal attainment was 45% and 18% in the high and very high risk groups, respectively. Use of statins in monotherapy was 24%, while 1% used statins in combination with other LLT. Sixty-two per cent had hypertension and approximately half of these patients were at BP goal. The majority of the patients used antihypertensive treatment in monotherapy (24%), while 10% and 5% as a two- or three-drug combination. Conclusion We revealed considerable geographical differences in estimated CVD risk and preventive treatment. Low goal attainment for LLT was observed, and only half the patients obtained BP goal. Despite a high focus on the increased CVD risk in RA patients over the last decade, there is still substantial potential for improvement in CVD preventive measures.

ObjectivePayments from the pharmaceutical industry to practicing physicians may influence prescribing behavior. This study was undertaken to investigate the nature, quantity, and geographic distribution of payments to US rheumatologists.MethodsGeneral payments from industry sponsors to US rheumatologists from 2014 to 2019 were extracted from the Centers for Medicare and Medicaid Services Open Payments database. Gender was identified by linking physicians to the National Plan and Provider Enumeration System registry. Data were reported in aggregate, trends over time were assessed using linear regression models, and differences by gender were analyzed using the Wilcoxon rank sum test.ResultsOver the 6‐year time period from 2014 to 2019, a total of 1,610,668 payments totaling $221,254,966 were made to 5,723 rheumatologists. The median payment was $15 (interquartile range [IQR] $10 to $22), and the median total amount received by individual rheumatologists over the 6‐year period was $2,818 (IQR $464 to $11,560). The majority of rheumatologists (3,416 of 5,723 [60%]) received less than $5,000, but 368 of 5,723 (6%) received more than $100,000 each and accounted for 78% of the total. The yearly value of payments increased over time ($3,703,264 per year; P < 0.001), and the median payment to male rheumatologists was significantly higher than the median payment to female rheumatologists ($3,723 [IQR $542 to $15,841] versus $2,084 [IQR $394 to $8,186]; P < 0.001).ConclusionThe value of industry payments has increased over time, and a large amount is concentrated among a small number of rheumatologists. Future studies should investigate the degree to which industry payments have influenced prescribing in the field of rheumatology.

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.

Background/Purpose Proprotein convertase subtilisin/kexin type 9 (PCSK9) is an enzyme that regulates cholesterol homeostasis by inducing posttranslational modification and lysosomal degradation of low‐density lipoprotein receptor (LDLR). However, the role of PCSK9 in dyslipidemia associated with rheumatoid arthritis (RA) is not well studied. The aim of this study was to examine how PCSK9 levels correlate with age [30–45; 46–60; and 61–80 years], sex, and RA disease, and with CD36, a major scavenger receptor targeted for proteasomal degradation by PCSK9. Methods De‐identified human RA serum samples (n=66; 33 female and 33 male) were obtained from patients diagnosed with RA according to the American College of Rheumatology 1987 classification criteria and healthy, age and gender‐matched serum samples were used for evaluation. Serum levels of human PCSK9 and CD36 were determined using commercially available ELISA kits. Results The mean (SD) age of healthy group [55.6 (11.2) years] and RA group [57.2 (10.4) years] was observed. Results of the ELISA showed that the serum levels of PCSK9 in RA group is reduced by ~10% (mean ± SEM; 231 ± 11.6 ng/ml vs. 205 ± 11.1 ng/ml; p<0.14). Interestingly, our gender‐based analysis showed that the basal serum PCSK9 levels were around 30% higher in the healthy female group compared to the healthy male group (260 ± 15.9 ng/ml vs. 202 ± 15.4 ng/ml; p=0.01). Furthermore, from a disease perspective, we observed approximately 15% lower serum PCSK9 levels in female RA group (260 ± 15.9 ng/ml vs. 219 ± 14.4 ng/ml; p=0.078) compared to only a modest 5% decline in male RA group (202 ± 15.4 ng/ml vs. 191 ± 16.8 ng/ml; p=0.62) when compared to their respective gender control groups. Analysis of PCSK9 data based on age showed that while PCSK9 levels gradually decreased in healthy female group [274.4 ± 38.9 ng/ml in 30–45y range vs. 241 ± 20.7 ng/ml in 61–80y], we observed an accelerated decrease in serum PCSK9 levels in RA female group [278.5 ± 62.4 ng/ml in 30–45y range vs. 220 ± 19.2 ng/ml in 46–60y; p=0.14]. This suggests that PCSK9 levels rapidly decline with RA progression in aging females. Interestingly, the serum PCSK9 levels in both healthy and RA male groups showed declines at a later stage of life [61–80y range; p<0.001], implicating that PCSK9 levels deplete in male groups, independent of RA disease pathogenesis. Serum CD36 levels in healthy female group were modestly low when compared to the serum levels in healthy male group [mean ± SEM; 1121 ± 164 pg/ml vs. 1280 ± 216 pg/ml; p<0.562]. Interestingly, while the CD36 levels were around 30% higher in RA males compared to the healthy male controls, we observed a modest decrease in serum CD36 by 15–20% in RA females when compared to the healthy female controls. Conclusion Our findings provide evidence of the gender differences in serum PCSK9 levels and its modulation with age. Furthermore, it is speculated that an early decline in serum PCSK9 in female RA patients may contribute towards the establishment of RA in females. Support or Funding Information Washington State University, Spokane, WA This abstract is from the Experimental Biology 2019 Meeting. There is no full text article associated with this abstract published in The FASEB Journal .