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77 Publications visible to you, out of a total of 77

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OBJECTIVE: Extensive studies on health-related quality of life (HRQoL) in idiopathic inflammatory myopathies (IIMs) are lacking. Our objective was to document HRQoL and to identify factors associated with a reduced HRQoL in patients with IIM. METHODS: A total of 1,715 patients (median age 49.9 years, 70% female, 87% white) who met probable or definite Bohan and Peter criteria or Griggs criteria for myositis were included from the Myovision registry. HRQoL was ascertained using the Short Form 12 (SF-12) health survey questionnaire. HRQoL physical component summary (PCS) and mental component summary (MCS) scores in relation to different patient and disease characteristics were compared to scores from matched normative data from the US general population and rheumatoid arthritis (RA) patients. Bivariate and multiple linear regression analyses were performed to assess the association between HRQoL and patient and disease parameters. RESULTS: The mean SF-12 summary scores were significantly lower in IIM patients than in the normative and RA populations. A diagnosis of inclusion body myositis, older age, patient-reported negative effect of disease on work, presence of another co-occurring autoimmune disease, polypharmacy, and IIM-associated lung disease and joint involvement were significantly associated with lower PCS scores. Lower MCS scores were associated with joint involvement and a negative effect of disease on work. CONCLUSION: In this large study of patient-reported outcomes in IIM, an association was found between multiple disease characteristics and reduced HRQoL, mostly in the physical domain. In the US, the HRQoL of IIM patients was found to be lower than that of the general population and RA patients.

Authors: M. Feldon, P. N. Farhadi, H. I. Brunner, L. Itert, B. Goldberg, A. Faiq, J. Wilkerson, K. M. Rose, L. G. Rider, F. W. Miller, E. H. Giannini

Date Published: 2017

Publication Type: Journal

Abstract (Expand)

OBJECTIVE: To develop and validate new classification criteria for adult and juvenile idiopathic inflammatory myopathies (IIM) and their major subgroups. METHODS: Candidate variables were assembled from published criteria and expert opinion using consensus methodology. Data were collected from 47 rheumatology, dermatology, neurology, and pediatric clinics worldwide. Several statistical methods were utilized to derive the classification criteria. RESULTS: Based on data from 976 IIM patients (74% adults; 26% children) and 624 non-IIM patients with mimicking conditions (82% adults; 18% children), new criteria were derived. Each item is assigned a weighted score. The total score corresponds to a probability of having IIM. Subclassification is performed using a classification tree. A probability cutoff of 55%, corresponding to a score of 5.5 (6.7 with muscle biopsy) "probable IIM," had best sensitivity/specificity (87%/82% without biopsies, 93%/88% with biopsies) and is recommended as a minimum to classify a patient as having IIM. A probability of >/=90%, corresponding to a score of >/=7.5 (>/=8.7 with muscle biopsy), corresponds to "definite IIM." A probability of <50%, corresponding to a score of <5.3 (<6.5 with muscle biopsy), rules out IIM, leaving a probability of >/=50-<55% as "possible IIM." CONCLUSION: The European League Against Rheumatism/American College of Rheumatology (EULAR/ACR) classification criteria for IIM have been endorsed by international rheumatology, dermatology, neurology, and pediatric groups. They employ easily accessible and operationally defined elements, and have been partially validated. They allow classification of "definite," "probable," and "possible" IIM, in addition to the major subgroups of IIM, including juvenile IIM. They generally perform better than existing criteria.

Authors: I. E. Lundberg, A. Tjarnlund, M. Bottai, V. P. Werth, C. Pilkington, M. de Visser, L. Alfredsson, A. A. Amato, R. J. Barohn, M. H. Liang, J. A. Singh, R. Aggarwal, S. Arnardottir, H. Chinoy, R. G. Cooper, K. Danko, M. M. Dimachkie, B. M. Feldman, I. Garcia-De La Torre, P. Gordon, T. Hayashi, J. D. Katz, H. Kohsaka, P. A. Lachenbruch, B. A. Lang, Y. Li, C. V. Oddis, M. Olesinska, A. M. Reed, L. Rutkowska-Sak, H. Sanner, A. Selva-O’Callaghan, Y. W. Song, J. Vencovsky, S. R. Ytterberg, F. W. Miller, L. G. Rider, the Euromyositis Register International Myositis Classification Criteria Project Consortium, Study the Juvenile Dermatomyositis Cohort Biomarker, Repository

Date Published: 2017

Publication Type: Journal

Abstract (Expand)

OBJECTIVE: To estimate the incidence and mortality rates, and their evolution over time, of physician-diagnosed primary Sjogren syndrome (pSS) in residents of Olmsted County, Minnesota. PATIENTS AND METHODS: Medical records of patients with a diagnosis or suspicion of SS in Olmsted County from January 1, 2006, through December 31, 2015, were reviewed to identify incident cases of pSS (defined by physician diagnosis). These cases were combined with those from a 1976 through 2005 incident cohort (n=111) from the same population. Incidence rates were age and sex adjusted to the 2010 US white population. Survival rates were compared with the expected rates in the population of Minnesota. RESULTS: With 61 incident cases of pSS diagnosed in Olmsted County from 2006 through 2015, the total cohort included 172 patients with incident pSS from 1976 through 2015. Of the 172 patients, 151 (88%) were women and 161 (94%) were white, with a mean +/- SD age at diagnosis of 58.3+/-16.7 years. The average age- and sex-adjusted annual incidence for 2006 through 2015 was 5.9 per 100,000 population (95% CI, 4.4-7.4 per 100,000 population), and the overall incidence for the entire period was 5.8 per 100,000 (95% CI, 4.9-6.6 per 100,000). The incidence increased with calendar time over the 40-year period (P=.005). There was no difference in mortality in the pSS cohort compared with expected (standardized mortality ratio, 1.15; 95% CI, 0.86-1.50). CONCLUSION: The average annual incidence of pSS in this population-based cohort was 5.8 per 100,000, with a progressive increase over the 40 years of the study. Overall survival of patients with pSS was not different from that of the general population.

Authors: G. Maciel, C. S. Crowson, E. L. Matteson, D. Cornec

Date Published: 2017

Publication Type: Journal

Abstract (Expand)

OBJECTIVE: To report the point prevalence of primary Sjogren’s syndrome (SS) in the first US population-based study. METHODS: Cases of all potential primary SS patients living in Olmsted County, Minnesota, on January 1, 2015, were retrieved using Rochester Epidemiology Project resources, and ascertained by manual medical records review. Primary SS cases were defined according to physician diagnosis. The use of diagnostic tests was assessed and the performance of classification criteria was evaluated. The number of prevalent cases in 2015 was also projected based on 1976-2005 incidence data from the same source population. RESULTS: A total of 106 patients with primary SS were included in the study: 86% were female, with a mean +/- SD age of 64.6 +/- 15.2 years, and a mean +/- SD disease duration of 10.5 +/- 8.4 years. A majority were anti-SSA positive (75%) and/or anti-SSB positive (58%), but only 22% met American-European Consensus Group or American College of Rheumatology criteria, because the other tests required for disease classification (ocular dryness objective assessment, salivary gland functional or morphologic tests, or salivary gland biopsy) were rarely performed in clinical practice. According to the physician diagnosis, the age- and sex-adjusted prevalence of primary SS was 10.3 per 10,000 inhabitants, but according to classification criteria, this prevalence was only 2.2 per 10,000. The analysis based on previous incidence data projected a similar 2015 prevalence rate of 11.0 per 10,000. CONCLUSION: The prevalence of primary SS in this geographically well-defined population was estimated to be between 2 and 10 per 10,000 inhabitants. Physicians rarely used tests included in the classification criteria to diagnose the disease in this community setting.

Authors: G. Maciel, C. S. Crowson, E. L. Matteson, D. Cornec

Date Published: 2017

Publication Type: Journal

Abstract (Expand)

The aim of the study was to evaluate the levels of physical activity in individuals with primary Sjogren’s syndrome (PSS) and its relationship to the clinical features of PSS. To this cross-sectional study, self-reported levels of physical activity from 273 PSS patients were measured using the International Physical Activity Questionnaire-short form (IPAQ-SF) and were compared with healthy controls matched for age, sex and body mass index. Fatigue and other clinical aspects of PSS including disease status, dryness, daytime sleepiness, dysautonomia, anxiety and depression were assessed using validated tools. Individuals with PSS had significantly reduced levels of physical activity [median (interquartile range, IQR) 1572 (594-3158) versus 3708 (1732-8255) metabolic equivalent of task (MET) x min/week, p < 0.001], but similar levels of sedentary activity [median (IQR) min 300 (135-375) versus 343 (223-433) (MET) x min/week, p = 0.532] compared to healthy individuals. Differences in physical activity between PSS and controls increased at moderate [median (IQR) 0 (0-480) versus 1560 (570-3900) MET x min/week, p < 0.001] and vigorous intensities [median (IQR) 0 (0-480) versus 480 (0-1920) MET x min/week, p < 0.001]. Correlation analysis revealed a significant association between physical activity and fatigue, orthostatic intolerance, depressive symptoms and quality of life. Sedentary activity did not correlate with fatigue. Stepwise linear regression analysis identified symptoms of depression and daytime sleepiness as independent predictors of levels of physical activity. Physical activity is reduced in people with PSS and is associated with symptoms of depression and daytime sleepiness. Sedentary activity is not increased in PSS. Clinical care teams should explore the clinical utility of targeting low levels of physical activity in PSS.

Authors: W. F. Ng, A. Miller, S. J. Bowman, E. J. Price, G. D. Kitas, C. Pease, P. Emery, P. Lanyon, J. Hunter, M. Gupta, I. Giles, D. Isenberg, J. McLaren, M. Regan, A. Cooper, S. A. Young-Min, N. McHugh, S. Vadivelu, R. J. Moots, D. Coady, K. MacKay, B. Dasgupta, N. Sutcliffe, M. Bombardieri, C. Pitzalis, B. Griffiths, S. Mitchell, S. T. Miyamoto, M. Trenell, U. K. Primary Sjogren’s Syndrome Registry

Date Published: 2017

Publication Type: Journal

Abstract (Expand)

OBJECTIVE: To define a set of core patient-reported domains and respective instruments for use in idiopathic inflammatory myopathies (IIM). Previously, we reported a systematic literature review on patient-reported outcomes (PRO) in IIM followed by conducting international focus groups to elicit patient perspectives of myositis symptoms and effects. METHODS: Based on qualitative content analysis of focus groups, an initial list of 26 candidate domains was constructed. We subsequently conducted an international modified Delphi survey to identify the importance of each of the 26 domains. Participants were asked to rate each domain on a scale of 0-10 (0 = not important, 10 = very important). RESULTS: In this first round of the Delphi survey, 643 patients participated from the United States (n = 543), Sweden (n = 49), and South Korea (n = 51). Of the 26 domains, 19 (73%) were rated of high importance (>/= 7/10). The top 5 domains were muscle symptoms, fatigue, interactions with healthcare, medication side effects, and pain. During Outcome Measures in Rheumatology (OMERACT) 2016, we discussed the goal for ultimate reduction in the number of domains and the importance of considering representation of healthcare providers from other specialties, caregivers, representatives of pharmaceutical industries, and regulatory authorities in the next rounds of Delphi to represent broader perspectives on IIM. CONCLUSION: Further prioritization and a reduction in the number of domains will be needed for the next Delphi. At the next biennial OMERACT meeting, we aim to present and seek voting on a Myositis Preliminary PRO Core Set to enable ultimate measure selection and development.

Authors: J. K. Park, C. A. Mecoli, H. Alexanderson, M. Regardt, L. Christopher-Stine, M. Casal-Dominguez, I. de Groot, C. Sarver, I. E. Lundberg, C. O. Bingham, Y. W. Song

Date Published: 2017

Publication Type: Journal

Abstract (Expand)

BACKGROUND AND AIMS: Biologics (disease modifying antirheumatic drugs, bDMARD) have been in use in Germany for the treatment of rheumatoid arthritis (RA) since 2001, usually after failure of at least one conventional synthetic (cs)DMARD. We analyzed temporal changes in factors that influence the decision for either a first bDMARD or a further csDMARD. MATERIAL AND METHODS: We analyzed data from 9513 bDMARD-naive RA patients in the German biologics register RABBIT who switched to a new therapy. For three recruitment periods (2001-2003, 2004-2006 and 2009-2015) factors influencing the therapeutic decision were analyzed by means of machine learning methods and logistic regression analysis. RESULTS: In all recruitment periods the number of previous csDMARDs, high dosages of glucocorticoids (>7.5 mg/day) and a higher DAS28 (>5.1) were significantly associated with the decision for a first bDMARD. Over time, the chance of receiving a bDMARD increased in patients with moderate disease activity, moderate glucocorticoid dosages (5-7.5 mg/day) and those with comorbidities, such as congestive heart failure or prior malignancy. Men had a higher chance of receiving a bDMARD than women only in the first recruitment period. Private health insurance, high education and gainful employment were significantly associated with more frequent prescription of bDMARDs in all recruitment periods. DISCUSSION: The time-dependent changes in the impact of disease activity, concomitant drugs, gender and comorbidity on the prescription of bDMARDs mirror the increasing therapeutic options and the growing experience in the application of the new substances in patients at higher risk. The influence of demographic and social factors may reflect safety concerns in patients at increased risk of adverse events but also the need to economize drug costs..

Authors: D. Pattloch, A. Richter, B. Manger, R. Dockhorn, L. Meier, H. P. Tony, A. Zink, A. Strangfeld

Date Published: 2017

Publication Type: Journal

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