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Showing papers in "Arthritis Care and Research in 2012"


Journal ArticleDOI
TL;DR: To update the American College of Rheumatology (ACR) 2000 recommendations for hip and knee osteoarthritis (OA) and develop new recommendations for hand OA.
Abstract: Objective To update the American College of Rheumatology (ACR) 2000 recommendations for hip and knee osteoarthritis (OA) and develop new recommendations for hand OA. Methods A list of pharmacologic and nonpharmacologic modalities commonly used to manage knee, hip, and hand OA as well as clinical scenarios representing patients with symptomatic hand, hip, and knee OA were generated. Systematic evidence-based literature reviews were conducted by a working group at the Institute of Population Health, University of Ottawa, and updated by ACR staff to include additions to bibliographic databases through December 31, 2010. The Grading of Recommendations Assessment, Development and Evaluation approach, a formal process to rate scientific evidence and to develop recommendations that are as evidence based as possible, was used by a Technical Expert Panel comprised of various stakeholders to formulate the recommendations for the use of nonpharmacologic and pharmacologic modalities for OA of the hand, hip, and knee. Results Both “strong” and “conditional” recommendations were made for OA management. Modalities conditionally recommended for the management of hand OA include instruction in joint protection techniques, provision of assistive devices, use of thermal modalities and trapeziometacarpal joint splints, and use of oral and topical nonsteroidal antiinflammatory drugs (NSAIDs), tramadol, and topical capsaicin. Nonpharmacologic modalities strongly recommended for the management of knee OA were aerobic, aquatic, and/or resistance exercises as well as weight loss for overweight patients. Nonpharmacologic modalities conditionally recommended for knee OA included medial wedge insoles for valgus knee OA, subtalar strapped lateral insoles for varus knee OA, medially directed patellar taping, manual therapy, walking aids, thermal agents, tai chi, self-management programs, and psychosocial interventions. Pharmacologic modalities conditionally recommended for the initial management of patients with knee OA included acetaminophen, oral and topical NSAIDs, tramadol, and intraarticular corticosteroid injections; intraarticular hyaluronate injections, duloxetine, and opioids were conditionally recommended in patients who had an inadequate response to initial therapy. Opioid analgesics were strongly recommended in patients who were either not willing to undergo or had contraindications for total joint arthroplasty after having failed medical therapy. Recommendations for hip OA were similar to those for the management of knee OA. Conclusion These recommendations are based on the consensus judgment of clinical experts from a wide range of disciplines, informed by available evidence, balancing the benefits and harms of both nonpharmacologic and pharmacologic modalities, and incorporating their preferences and values. It is hoped that these recommendations will be utilized by health care providers involved in the management of patients with OA.

2,615 citations


Journal ArticleDOI
TL;DR: The 2012 ACR RA recommendations were developed by two expert panels: a non-voting working group and Core Expert Panel of clinicians and methodologists responsible for the selection of the relevant topic areas to be considered, the systematic literature review, and the evidence synthesis.
Abstract: The American College of Rheumatology (ACR) most recently published recommendations for use of disease modifying anti-rheumatic drugs (DMARDs) and biologics in the treatment of rheumatoid arthritis (RA) in 2008 (1). These recommendations covered indications for use, monitoring of side-effects, assessment of the clinical response to DMARDs and biologics, screening for tuberculosis (TB), and assessment of the roles of cost and patient preference in decision-making for biologic agents (1). Recognizing the rapidly evolving knowledge in RA management and the accumulation of new evidence regarding the safety and efficacy of existing and newer therapies, the ACR commissioned an update of the 2008 recommendations in select topic areas. The 2012 revision updates the 2008 ACR recommendations in the following areas: (1) indications for DMARDs and biologics; (2) switching between DMARD and biologic therapies; (3) use of biologics in high-risk patients (those with hepatitis, congestive heart failure, and malignancy); (4) screening for TB in patients starting or currently receiving biologics; and (5) vaccination in patients starting or currently receiving DMARDs or biologics (Table 1). Table 1 Overview Comparison of Topics and Medications Included in the 2008 and 2012 ACR RA Recommendations METHODS We utilized the same methodology as described in detail in the 2008 guidelines (1) to maintain consistency and to allow cumulative evidence to inform this 2012 recommendation update. These recommendations were developed by two expert panels: (1) a non-voting working group and Core Expert Panel (CEP) of clinicians and methodologists responsible for the selection of the relevant topic areas to be considered, the systematic literature review, and the evidence synthesis and creation of “clinical scenarios”; and (2) a Task Force Panel (TFP) of 11 internationally-recognized expert clinicians, patient representatives and methodologists with expertise in RA treatment, evidence-based medicine and patient preferences who were tasked with rating the scenarios created using an ordinal scale specified in the Research and Development/University of California at Los Angeles (RAND/UCLA) Appropriateness method (2–4). This method solicited formal input from a multi-disciplinary TFP panel to make recommendations informed by the evidence. The methods used to develop the updated ACR recommendations are described briefly below. Systematic Literature Review – Sources, Databases and Domains Literature searches for both DMARDs and biologics relied predominantly on PubMed searches) with medical subject headings (MeSH) and relevant keywords similar to those used for the 2008 ACR RA recommendations (see Appendices 1 and 2). We included randomized clinical trials (RCTs), controlled clinical trials (CCTs), quasi-experimental designs, cohort studies (prospective or retrospective), and case-control studies, with no restrictions on sample size. More details about inclusion criteria are listed below and in Appendix 3. The 2008 recommendations were based on a literature search that ended on February 14, 2007. The literature search end date for the 2012 Update was February 26, 2010 for the efficacy and safety studies and September 22, 2010 for additional qualitative reviews related to TB screening, immunization and hepatitis (similar to the 2008 methodology). Studies published subsequent to that date were not included. For biologics, we also reviewed the Cochrane systematic reviews and overviews (published and in press) in the Cochrane Database of Systematic Reviews to identify additional studies (5–8) and further supplemented by hand-checking the bibliographies of all included articles. Finally, the CEP and TFP confirmed that relevant literature was included for evidence synthesis. Unless they were identified by the literature search and met the article inclusion criteria (see Appendix 3), we did not review any unpublished data from product manufacturers, investigators, or the Food and Drug Administration (FDA) Adverse Event Reporting System. We searched the literature for the eight DMARDs and nine biologics most commonly used for the treatment of RA. Literature was searched for eight DMARDS including azathioprine, cyclosporine, hydroxychloroquine, leflunomide, methotrexate, minocycline, organic gold compounds and sulfasalazine. As in 2008, azathioprine, cyclosporine and gold were not included in the recommendations based on infrequent use and lack of new data (Table 1). Literature was searched for nine biologics including abatacept, adalimumab, anakinra, certolizumab pegol, etanercept, golimumab, infliximab, rituximab and tocilizumab; anakinra was not included in the recommendations due to infrequent use and lack of new data. Details of the bibliographic search strategy are listed in Appendix 1.

1,493 citations


Journal ArticleDOI
TL;DR: DINESH KHanNA, JOHN D. FITZGERALD, PUJA P. KHANNA, SANGMEE BAE, MANJIT K. SINGH, TUHINA NEOGI, MICHAEL H. PILLINGER, JOAN MERILL, SUSAN LEE, SHRADDHA PRAKASH, MARIAN KALDAS, MANEESH GOGIA, FERNANDO PEREZ-RUI
Abstract: DINESH KHANNA, JOHN D. FITZGERALD, PUJA P. KHANNA, SANGMEE BAE, MANJIT K. SINGH, TUHINA NEOGI, MICHAEL H. PILLINGER, JOAN MERILL, SUSAN LEE, SHRADDHA PRAKASH, MARIAN KALDAS, MANEESH GOGIA, FERNANDO PEREZ-RUIZ, WILL TAYLOR, FREDERIC LIOTE, HYON CHOI, JASVINDER A. SINGH, NICOLA DALBETH, SANFORD KAPLAN, VANDANA NIYYAR, DANIELLE JONES, STEVEN A. YAROWS, BLAKE ROESSLER, GAIL KERR, CHARLES KING, GERALD LEVY, DANIEL E. FURST, N. LAWRENCE EDWARDS, BRIAN MANDELL, H. RALPH SCHUMACHER, MARK ROBBINS, NEIL WENGER, AND ROBERT TERKELTAUB

1,335 citations


Journal ArticleDOI
TL;DR: New classification criteria for Sjögren's syndrome are proposed, which are needed considering the emergence of biologic agents as potential treatments and their associated comorbidity.
Abstract: Objectives We propose new classification criteria for Sjogren’s Syndrome (SS), which are needed considering the emergence of biological agents as potential treatments and their associated co-morbidity. These criteria target individuals with signs/symptoms suggestive of SS.

1,147 citations


Journal ArticleDOI
TL;DR: The management strategies discussed here apply to lupus nephritis in adults, particularly to those receiving care in the United States of America, and include interventions that were available in theUnited States as of April 2011.
Abstract: In the United States, approximately 35% of adults with Systemic Lupus Erythematosus (SLE) have clinical evidence of nephritis at the time of diagnosis; with an estimated total of 50–60% developing nephritis during the first 10 years of disease [1–4]. The prevalence of nephritis is significantly higher in African Americans and Hispanics than in Caucasians, and is higher in men than in women. Renal damage is more likely to develop in non-Caucasian groups [2–4]. Overall survival in patients with SLE is approximately 95% at 5 years after diagnosis and 92% at 10 years [5, 6]. The presence of lupus nephritis significantly reduces survival, to approximately 88% at 10 years, with even lower survival in African Americans [5, 6]. The American College of Rheumatology (ACR) last published guidelines for management of systemic lupus erythematosus (SLE) in 1999 [7]. That publication was designed primarily for education of primary care physicians and recommended therapeutic and management approaches for many manifestations of SLE. Recommendations for management of lupus nephritis (LN) consisted of pulse glucocorticoids followed by high dose daily glucocorticoids in addition to an immunosuppressive medication, with cyclophosphamide viewed as the most effective immunosuppressive medication for diffuse proliferative glomerulonephritis. Mycophenolate mofetil was not yet in use for lupus nephritis and was not mentioned. Since that time, many clinical trials of glucocorticoids-plus-immunosuppressive interventions have been published, some of which are high quality prospective trials, and some not only prospective but also randomized. Thus, the ACR determined that a new set of management recommendations was in order. A combination of extensive literature review and the opinions of highly qualified experts, including rheumatologists, nephrologists and pathologists, has been used to reach the recommendations. The management strategies discussed here apply to lupus nephritis in adults, particularly to those receiving care in the United States of America, and include interventions that were available in the United States as of April 2011. While these recommendations were developed using rigorous methodology, guidelines do have inherent limitations in informing individual patient care; hence the selection of the term “recommendations.” While they should not supplant clinical judgment or limit clinical judgment, they do provide expert advice to the practicing physician managing patients with lupus nephritis.

1,128 citations


Journal ArticleDOI
TL;DR: The American College of Rheumatology as mentioned in this paper conducted a systematic review of the literature to identify rheumatoid arthritis (RA) disease activity measures using exclusion criteria, input from an Expert Advisory Panel and psychometric analysis, a list of potential measures was created.
Abstract: Guidelines and recommendations developed and/or endorsed by the American College of Rheumatology (ACR) are intended to provide guidance for particular patterns of practice and not to dictate the care of a particular patient. The ACR considers adherence to these guidelines and recommendations to be voluntary, with the ultimate determination regarding their application to be made by the physician in light of each patient’s individual circumstances. Guidelines and recommendations are intended to promote beneficial or desirable outcomes but cannot guarantee any specific outcome. Guidelines and recommendations developed or endorsed by the ACR are subject to periodic revision as warranted by the evolution of medical knowledge, technology, and practice. The American College of Rheumatology is an independent, professional, medical and scientific society which does not guarantee, warrant, or endorse any commercial product or service. Objective. Although the systematic measurement of disease activity facilitates clinical decision making in rheumatoid arthritis (RA), no recommendations currently exist on which measures should be applied in clinical practice in the US. The American College of Rheumatology (ACR) convened a Working Group (WG) to comprehensively evaluate the validity, feasibility, and acceptability of available RA disease activity measures and derive recommendations for their use in clinical practice. Methods. The Rheumatoid Arthritis Clinical Disease Activity Measures Working Group conducted a systematic review of the literature to identify RA disease activity measures. Using exclusion criteria, input from an Expert Advisory Panel (EAP), and psychometric analysis, a list of potential measures was created. A survey was administered to rheumatologists soliciting input. The WG used these survey results in conjunction with the psychometric analyses to derive final recommendations. Results. Systematic review of the literature resulted in identification of 63 RA disease activity measures. Application of exclusion criteria and ratings by the EAP narrowed the list to 14 measures for further evaluation. Practicing rheumatologists rated 9 of these 14 measures as most useful and feasible. From these 9 measures, the WG selected 6 with the best psychometric properties for inclusion in the final set of ACR-recommended RA disease activity measures. Conclusion. We recommend the Clinical Disease Activity Index, Disease Activity Score with 28-joint counts (erythrocyte sedimentation rate or C-reactive protein), Patient Activity Scale (PAS), PAS-II, Routine Assessment of Patient Index Data with 3 measures, and Simplified Disease Activity Index because they are accurate reflections of disease activity; are sensitive to change; discriminate well between low, moderate, and high disease activity states; have remission criteria; and are feasible to perform in clinical settings.

608 citations


Journal ArticleDOI
TL;DR: These guidelines for the management and anti-inflammatory prophylaxis of acute attacks of gouty arthritis complements the manuscript on guidelines to treat hyperuricemia in patients with evidence of gouts and involves a diverse and international panel of experts.
Abstract: In response to a request for proposal from the American College of Rheumatology (ACR), our group was charged with developing non-pharmacologic and pharmacologic guidelines for treatments in gout that are safe and effective, i.e., with acceptable risk-benefit ratio. These guidelines for the management and anti-inflammatory prophylaxis of acute attacks of gouty arthritis complements our manuscript on guidelines to treat hyperuricemia in patients with evidence of gout (or gouty arthritis) (1). Gout is the most common cause of inflammatory arthritis in adults in the USA. Clinical manifestations in joints and bursa are superimposed on top of local deposition of monosodium urate crystals. Acute gout characteristically presents as self-limited, attack of synovitis (also called “gout flares”). Acute gout attacks account for a major component of the reported decreased health-related quality of life in patients with gout (2, 3). Acute gout attacks can be debilitating and are associated with decreased work productivity (4, 5). Urate lowering therapy (ULT) is a cornerstone in the management of gout, and, when effective in lowering serum urate (SUA), is associated with decreased risk of acute gouty attacks (6). However, during the initial phase of ULT, there is an early increase in acute gout attacks, which has been hypothesized due to remodeling of articular urate crystal deposits as a result of rapid and substantial lowering of ambient urate concentrations (7). Acute gout attacks attributable to the initiation of ULT may contribute to non-adherence in long-term gout treatment, as reported in recent studies (8). In order to systematically evaluate a broad spectrum of acute gouty arthritis, we generated multifaceted case scenarios to elucidate decision making based primarily on clinical and laboratory test-based data that can be obtained in a gout patient by both non-specialist and specialist health care providers in an office practice setting. This effort was not intended to create a novel classification system of gout, or new gout diagnostic criteria, as such endeavors are beyond the scope of this work. Prior gout recommendations and guidelines, at the independent (i.e, non pharmaceutical industry-sponsored) national or multinational rheumatology society level, have been published by EULAR (9, 10), the Dutch College of General Practitioners (11), and the British Society for Rheumatology (BSR)(12). The ACR requested new guidelines, in view of the increasing prevalence of gout (13), the clinical complexity of management of gouty arthritis imposed by co-morbidities common in gout patients (14), and increasing numbers of treatment options via clinical development of agents(15–17). The ACR charged us to develop these guidelines to be useful for both rheumatologists and other health care providers on an international level. As such, this process and resultant recommendations, involved a diverse and international panel of experts. In this manuscript, we concentrate on 2 of the 4 gout domains that the ACR requested for evaluation of pharmacologic and non-pharmacologic management approaches: (i) analgesic and anti-inflammatory management of acute attacks of gouty arthritis, and (ii) pharmacologic anti-inflammatory prophylaxis of acute attacks of gouty arthritis. Part I of the guidelines focused on systematic non-pharmacologic measures (patient education, diet and lifestyle choices, identification and management of co-morbidities) that impact on hyperuricemia, and made recommendations on pharmacologic ULT in a broad range of case scenarios of patients with disease activity manifested by acute and chronic forms of gouty arthritis, including chronic tophaceous gouty arthropathy(1). Each individual and specific statement is designated as a “recommendation”, in order to reflect the non-prescriptive nature of decision making for the hypothetical clinical scenarios. So that the voting panel could focus on gout treatment decisions, a number of key assumptions were made, as described in Part I of the guidelines (1). Importantly, each proposed recommendation assumed that correct diagnoses of gout and acute gouty arthritis attacks had been made for the voting scenario in question. For treatment purposes, it was also assumed that treating clinicians were competent, and considered underlying medical comorbidities (including diabetes, gastrointestinal disease, hypertension, and hepatic, cardiac, and renal disease), and potential drug toxicities and drug-drug interactions, when making both treatment choicesand dosing decisions on chosen pharmacologic interventions. The RAND/UCLA methodology used here emphasizes level of evidence, safety, and quality of therapy, and excludes analyses of societal cost of health care. As such, the ACR gout guidelines are designed to reflect best practice, supported either by level of evidence or consensus-based decision-making. These guidelines cannot substitute for individualized, direct assessment of the patient, coupled with clinical decision making by a competent health care practitioner. The motivation, financial circumstances, and preferences of the gout patient also need to be considered in clinical practice, and it is incumbent on the treating clinician to weigh the issues not addressed by this methodology, such as treatment costs, when making management decisions. Last, the guidelines for gout management presented herein were not designed to determine eligibility for health care cost coverage by third party payers.

574 citations


Journal ArticleDOI
TL;DR: To estimate the minimum clinically important improvement (MCII) and patient acceptable symptom state (PASS) values for 4 generic outcomes in 5 rheumatic diseases and 7 countries, a database of more than 2,000 patients in 7 countries was used.
Abstract: Objective To estimate the minimum clinically important improvement (MCII) and patient acceptable symptom state (PASS) values for 4 generic outcomes in 5 rheumatic diseases and 7 countries. Methods We conducted a multinational (Australia, France, Italy, Lebanon, Morocco, Spain, and The Netherlands) 4-week cohort study involving 1,532 patients who were prescribed nonsteroidal antiinflammatory drugs for ankylosing spondylitis, chronic back pain, hand osteoarthritis, hip and/or knee osteoarthritis, or rheumatoid arthritis. The MCII and PASS values were estimated with the 75th percentile approach for 4 generic outcomes: pain, patient global assessment, functional disability, and physician global assessment, all normalized to a 0-100 score. Results For the whole sample, the estimated MCII values for absolute change at 4 weeks were -17 (95% confidence interval [95% CI] -18, -15) for pain; -15 (95% CI -16, -14) for patient global assessment; -12 (95% CI -13, -11) for functional disability assessment; and -14 (95% CI -15, -14) for physician global assessment. For the whole sample, the estimated PASS values were 42 (95% CI 40, 44) for pain; 43 (95% CI 41, 45) for patient global assessment; 43 (95% CI 41, 44) for functional disability assessment; and 39 (95% CI 37, 40) for physician global assessment. Estimates were consistent across diseases and countries (for subgroups ≥20 patients). Conclusion This work allows for promoting the use of values of MCII (15 of 100 for absolute improvement, 20% for relative improvement) and PASS (40 of 100) in reporting the results of trials of any of the 5 involved rheumatic diseases with pain, patient global assessment, physical function, or physician global assessment used as outcome criteria.

299 citations


Journal ArticleDOI
TL;DR: Patients with axial spondylarthritis who have structural changes in the sacroiliac joints and/or the spine have been classified as having ankylosing spondYLitis (AS), while those without such changes are nowclassified as having nonradiographic axial SpA (nr‐axSpA).
Abstract: Objective Patients with axial spondylarthritis (SpA) who have structural changes in the sacroiliac joints and/or the spine have been classified as having ankylosing spondylitis (AS), while those without such changes are now classified as having nonradiographic axial SpA (nr-axSpA). The differentiating features are incompletely understood. Methods Data from 100 consecutive patients with axial SpA not treated with tumor necrosis factor antagonists were compared clinically and with laboratory parameters, spinal radiographs, and magnetic resonance imaging (MRI) of the spine. Standardized clinical assessment tools were used to assess health status. Results AS was diagnosed in 56 patients and nr-axSpA in 44 patients. Signs of inflammation were significantly higher in patients with AS than in patients with nr-axSpA, with a median C-reactive protein level of 8.0 versus 3.8 mg/liter, a median Ankylosing Spondylitis Disease Activity Score of 2.2 versus 2.8, respectively, and a median amount of spinal inflammatory lesions on MRI of 2.0 versus 0.0, respectively. Significant differences between these 2 groups were seen in sex (76.8% male AS patients versus 31.8% male nr-axSpA patients). Clinical variables did not differ between patients with AS and nr-axSpA (Bath Ankylosing Spondylitis Disease Activity Index, Bath Ankylosing Spondylitis Functional Index, Ankylosing Spondylitis Quality of Life questionnaire, Short Form 36 health survey). Conclusion Patients with nr-axSpA were characterized by the low proportion of male patients and the low burden of inflammation compared to patients with AS. While both groups did not differ regarding health status, disease activity, and physical function, they did differ in signs of inflammation; all were higher in patients with AS. Since many patients with nr-axSpA had not developed structural changes after years of symptoms, we propose that those patients should not be regarded as having preradiographic AS but rather as having nr-axSpA.

284 citations


Journal ArticleDOI
TL;DR: Prevalence, severity, and outcome across countries in the Asia‐Pacific region of systemic lupus erythematosus is described.
Abstract: Objective Systemic lupus erythematosus (SLE), a chronic multisystem autoimmune disease with a wide spectrum of manifestations, shows considerable variation across the globe, although there is little evidence to indicate its relative prevalence in Asia. This review describes its prevalence, severity, and outcome across countries in the Asia-Pacific region. Methods We conducted a systematic literature search using 3 groups of terms (SLE, epidemiology, and Asia-Pacific countries) of EMBase and PubMed databases and non–English language resources, including Chinese Wanfang, Korean KMbase, Korean College of Rheumatology, Japana Centra Revuo Medicina, Taiwan National Digital Library of Theses and Dissertations, and Taiwanese, Thai, and Vietnamese journals. Results The review showed considerable variation in SLE burden and survival rates across Asia-Pacific countries. Overall crude incidence rates (per 100,000 per year) ranged from 0.9–3.1, while crude prevalence rates ranged from 4.3–45.3 (per 100,000). Higher rates of renal involvement, one of the main systems involved at death, were observed for Asians (21–65% at diagnosis and 40–82% over time) than for whites. While infections and active SLE were leading causes of death, a substantial proportion (6–40%) of deaths was due to cardiovascular involvement. The correlation between the Human Development Index and 5-year survival was 0.83. Conclusion This review highlights the need to closely monitor Asian SLE patients in Asian countries for renal and cardiovascular involvement, especially those who may not receive proper treatment and are therefore at greater risk of severe disease. We hope this will encourage further research specific to this region and lead to improved clinical management.

263 citations


Journal ArticleDOI
TL;DR: The outcomes of 10 patients with relapsing/refractory GCA, TA, or PMR treated with tocilizumab (TCZ) are assessed.
Abstract: Objective The interleukin-6 pathway is up-regulated in giant cell arteritis (GCA), Takayasu arteritis (TA), and polymyalgia rheumatica (PMR). We retrospectively assessed the outcomes of 10 patients with relapsing/refractory GCA, TA, or PMR treated with tocilizumab (TCZ). Methods Patients with GCA (n = 7), TA (n = 2), and PMR (n = 1) received TCZ. Seven subjects had failed at least 1 second-line agent. The outcomes evaluated were symptoms of disease activity, inflammatory markers, ability to taper glucocorticoids, and cross-sectional imaging when indicated clinically. Results The mean followup time of this cohort since diagnosis was 27 months (range 16–60 months). The patients were treated with TCZ for a mean period of 7.8 months (range 4–12 months). Before TCZ therapy, the patients experienced an average of 2.4 flares/year. All patients entered and maintained clinical remission during TCZ therapy. The mean daily prednisone dosages before and after TCZ initiation were 20.8 mg/day (range 7–34.3 mg/day) and 4.1 mg/day (range 0–10.7 mg/day), respectively (P = 0.0001). The mean erythrocyte sedimentation rate declined from 41.5 mm/hour (range 11–68 mm/hour) to 7 mm/hour (range 2.2–11.3 mm/hour; P = 0.0001). The adverse effects of TCZ included mild neutropenia (n = 4) and transaminitis (n = 4). One patient flared 2 months after TCZ discontinuation. An autopsy on 1 patient who died from a postoperative myocardial infarction following elective surgery revealed persistent vasculitis of large and medium-sized arteries. Conclusion TCZ therapy led to clinical and serologic improvement in patients with refractory/relapsing GCA, TA, or PMR. The demonstration of persistent large-vessel vasculitis at autopsy of 1 patient who had shown a substantial response requires close scrutiny in larger studies.

Journal ArticleDOI
TL;DR: To evaluate the occurrence of emergency department (ED) visits due to humerus fractures in the US, a large number of patients are referred to the hospital based on a history of previous hip fractures.
Abstract: OBJECTIVE: The objective of this study was to evaluate the occurrence of emergency department (ED) visits due to humerus fractures in the United States (U.S.). METHODS: We analyzed the 2008 Nationwide Emergency Department Sample, which contained approximately 28 million ED records. We identified the cases of interest using diagnostic codes for proximal, shaft, and distal humerus fractures. RESULTS: In 2008, approximately 370,000 ED visits in the U.S. resulted from humerus fractures. Proximal humerus fractures were the most common, accounting for 50% of humerus fractures. The incidence rate of proximal humerus fractures followed the shape of an exponential function in the ages 40-84 for women (R(2) =97.9%) and 60-89 for men (R(2) =98.2%). After the exponential increase in these age intervals, the growth rate of proximal humerus fracture slowed and eventually decreased. The peak occurrence of distal humerus fractures was in children aged 5 to 9 years; yet, elderly women had an increased risk. As the baby boomer generation ages, unless fracture prevention programs improve, more than 490,000 ED visits are expected due to humerus fractures in 2030 when the youngest of the baby boomers turn 65 years old. CONCLUSIONS: Compared to epidemiologic studies in Japan and European countries, the incidence rates of humerus fractures are substantially higher in the U.S. The high incidence rate of humerus fractures in the expanding elderly population may contribute to the recent trend of rapid increase in shoulder arthroplasty in the U.S. Rigorous safety measures to reduce falls and improved preventive treatments of osteoporosis are needed. © 2011 by the American College of Rheumatology. Language: en

Journal ArticleDOI
TL;DR: The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans and standardized assessment schedules for use in clinical practice to facilitate such studies.
Abstract: Objective There is wide variation in therapeutic approaches to systemic juvenile idiopathic arthritis (JIA) among North American rheumatologists. Understanding the comparative effectiveness of the diverse therapeutic options available for treatment of systemic JIA can result in better health outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans and standardized assessment schedules for use in clinical practice to facilitate such studies. Methods Case-based surveys were administered to CARRA members to identify prevailing treatments for new-onset systemic JIA. A 2-day consensus conference in April 2010 employed modified nominal group technique to formulate preliminary treatment plans and determine important data elements for collection. Followup surveys were employed to refine the plans and assess clinical acceptability. Results The initial case-based survey identified significant variability among current treatment approaches for new-onset systemic JIA, underscoring the utility of standardized plans to evaluate comparative effectiveness. We developed 4 consensus treatment plans for the first 9 months of therapy, as well as case definitions and clinical and laboratory monitoring schedules. The 4 treatment regimens included glucocorticoids only, or therapy with methotrexate, anakinra, or tocilizumab, with or without glucocorticoids. This approach was approved by >78% of the CARRA membership. Conclusion Four standardized treatment plans were developed for new-onset systemic JIA. Coupled with data collection at defined intervals, use of these treatment plans will create the opportunity to evaluate comparative effectiveness in an observational setting to optimize initial management of systemic JIA.

Journal ArticleDOI
TL;DR: The US national prevalence of spondylarthritis (SpA) was estimated for 2 published sets of classification criteria: the Amor criteria and the European Spondylarthropathy Study Group (ESSG) criteria.
Abstract: Objective The US national prevalence of spondylarthritis (SpA) was estimated for 2 published sets of classification criteria: the Amor criteria and the European Spondylarthropathy Study Group (ESSG) criteria. These 2 SpA criteria sets have been the most widely utilized in previous population-based studies of SpA. Methods The US SpA prevalence estimates were based on a representative sample of 5,013 US adults ages 20–69 years who were examined in the US National Health and Nutrition Examination Survey (NHANES) 2009–2010. Results The overall age-adjusted prevalence of definite and probable SpA by the Amor criteria was 0.9% (95% confidence interval [95% CI] 0.7–1.1%), corresponding to an estimated 1.7 million persons (95% CI 1.4–2.1 million persons). The age-adjusted prevalence of SpA by the ESSG criteria was 1.4% (95% CI 1.0–1.9%), corresponding to an estimated 2.7 million persons (95% CI 1.9–3.7 million persons). There were no statistically significant sex differences in SpA prevalence. The SpA prevalence among non-Hispanic white persons was 1.0% (95% CI 0.7–1.5%) by the Amor criteria and 1.5% (95% CI 1.0–2.3%) by the ESSG criteria. SpA prevalence could not be reliably estimated in other race/ethnicity subgroups due to sample size imitations. Conclusion The SpA prevalence estimates are in the range of SpA prevalence estimates reported elsewhere in population-based surveys and it is likely that SpA may affect up to 1% of US adults, a prevalence similar to that reported for rheumatoid arthritis. The current US SpA prevalence estimates may be lower than the true value because the NHANES 2009–2010 data collection did not capture a complete set of the elements specified in the 2 SpA criteria sets.

Journal ArticleDOI
TL;DR: Assessment of the prevalence of anti‐HMGCR antibodies in groups of statin users with and without self‐limited musculoskeletal side effects found that these antibodies have diagnostic utility.
Abstract: Objective Statins, among the most commonly prescribed medications, are associated with a wide range of musculoskeletal side effects. These include a progressive autoimmune myopathy with anti–3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMGCR) antibodies that requires immunosuppression. However, it remains unknown whether these antibodies are found in statin users with and without self-limited musculoskeletal side effects; this limits their diagnostic utility. The current work assessed the prevalence of anti-HMGCR antibodies in these groups of statin users. Methods We determined the prevalence of anti-HMGCR antibodies in 1,966 participants (including 763 current statin users) in a substudy of the community-based Atherosclerosis Risk in Communities (ARIC) Study and 98 French Canadian subjects with familial hypercholesterolemia, including 51 with documented statin intolerance. Results No participant in the ARIC substudy, including those with past or current statin exposure at the time of sample collection, had anti-HMGCR antibodies. Similarly, none of 51 patients with self-limited statin intolerance or 47 statin-tolerant patients receiving maximal statin therapy were anti-HMGCR positive. Conclusion The majority of patients with and without statin exposure, including those with self-limited statin intolerance, do not develop anti-HMGCR antibodies. Therefore, anti-HMGCR antibodies are highly specific for those with an autoimmune myopathy.

Journal ArticleDOI
TL;DR: To investigate the association of anti–hydroxymethylglutaryl‐coenzyme A reductase (anti‐HMGCR) myopathy with HLA class I and II antigens, a large number of patients with confirmed HLA infections are diagnosed with central giant cell granuloma.
Abstract: Objective To investigate the association of anti–hydroxymethylglutaryl-coenzyme A reductase (anti-HMGCR) myopathy with HLA class I and II antigens. Methods HLA antigens were determined in 1) 20 white and 8 African American anti-HMGCR patients, 2) 487 white and 167 African American controls, and 3) 51 white subjects with mild self-limited statin intolerance. Results White anti-HMGCR patients had a higher frequency of the combination HLA–DR11, DQA5, and DQB7 than controls or statin-intolerant subjects (70% versus 17%; odds ratio [OR] 11.7 [95% confidence interval (95% CI) 4.0–35.3], P = 4.1 × 10−7 and 70% versus 21%; OR 8.3 [95% CI 2.2–33.9], P = 5.4 × 10−4, respectively). This combination was not increased in African American anti-HMGCR subjects compared to controls (13% versus 3%; OR 4.6 [95% CI 0.2–53.3], P = 0.2). However, DR11 was increased in African American anti-HMGCR patients compared to controls (88% versus 21%; OR 26.4 [95% CI 3.1–590.3], P = 0.0002). High-resolution mapping showed that 95% with DR11 had DRB1*11:01. DQA1 and DQB6 were less frequent in white anti-HMGCR–positive patients compared to controls (25% versus 65%; OR 0.2 [95% CI 0.1–0.5], P = 5.5 × 10−4 and 0% versus 45%; OR 0.0 [95% CI 0.0–0.3], P = 2.1 × 10−5, respectively). DRB11 was not associated with particular disease features. Conclusion DRB1*11:01 is associated with an increased risk of anti-HMGCR myopathy in whites and African Americans. These findings suggest a mechanistic link between statin exposure, increased HMGCR expression, and the possible presentation of HMGCR-derived peptide(s) by DRB1*11:01.

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TL;DR: The study objective was to estimate the prevalence and correlates of anxiety and depression among US adults with doctor‐diagnosed arthritis.
Abstract: Objective There has been limited characterization of the burden of anxiety and depression, especially the former, among US adults with arthritis in the general population. The study objective was to estimate the prevalence and correlates of anxiety and depression among US adults with doctor-diagnosed arthritis. Methods The study sample comprised US adults ages ≥45 years with doctor-diagnosed arthritis (n = 1,793) from the Arthritis Conditions Health Effects Survey (a cross-sectional, population-based, random-digit–dialed telephone interview survey). Anxiety and depression were measured using separate and validated subscales of the Arthritis Impact Measurement Scales. Prevalence was estimated for the sample overall and stratified by subgroups. Associations between correlates and each condition were estimated with prevalence ratios and 95% confidence intervals using logistic regression models. Results Anxiety was more common than depression (31% and 18%, respectively); overall, one-third of respondents reported at least 1 of the 2 conditions. Most (84%) of those with depression also had anxiety. Multivariable logistic regression modeling failed to identify a distinct profile of characteristics of those with anxiety and/or depression. Only half of the respondents with anxiety and/or depression had sought help for their mental health condition in the past year. Conclusion Despite the clinical focus on depression among people with arthritis, anxiety was almost twice as common as depression. Given their high prevalence, their profound impact on quality of life, and the range of effective treatments available, we encourage health care providers to screen all people with arthritis for both anxiety and depression.

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TL;DR: This work investigated the long‐term clinical and cost effectiveness of a rehabilitation program combining self‐management and exercise: Enabling Self‐Management and Coping of Arthritic Knee Pain Through Exercise (ESCAPE‐knee pain).
Abstract: Chronic joint pain is a major cause of pain and disability. Exercise and self-management have short-term benefits, but few studies follow participants for more than 6 months. We investigated the long-term (up to 30 months) clinical and cost effectiveness of a rehabilitation program combining self-management and exercise: Enabling Self- Management and Coping of Arthritic Knee Pain Through Exercise (ESCAPE-knee pain). Methods. In this pragmatic, cluster randomized, controlled trial, 418 people with chronic knee pain (recruited from 54 primary care surgeries) were randomized to usual care (pragmatic control) or the ESCAPE-knee pain program. The primary outcome was physical function (Western Ontario and McMaster Universities Osteoarthritis Index [WOMAC] function), with a clinically meaningful improvement in physical function defined as a ≥15% change from baseline. Secondary outcomes included pain, psychosocial and physiologic variables, costs, and cost effectiveness. Results. Compared to usual care, ESCAPE-knee pain participants had large initial improvements in function (mean difference in WOMAC function -5.5; 95% confidence interval [95% CI] -7.8, -3.2). These improvements declined over time, but 30 months after completing the program, ESCAPE-knee pain participants still had better physical function (difference in WOMAC function -2.8; 95% CI -5.3, -0.2); lower community-based health care costs (£-47; 95% CI £-94, £-7), medication costs (£-16; 95% CI £-29, £-3), and total health and social care costs (£-1,118; 95% CI £-2,566, £-221); and a high probability (80-100%) of being cost effective. Conclusion. Clinical and cost benefits of ESCAPE-knee pain were still evident 30 months after completing the program. ESCAPE-knee pain is a more effective and efficient model of care that could substantially improve the health, well-being, and independence of many people, while reducing health care costs. © 2012, American College of Rheumatology.

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TL;DR: A recently developed multibiomarker disease activity (MBDA) test relative to clinical disease activity in diverse RA cohorts is validated and aid rheumatologists in clinical decision making.
Abstract: Objective Quantitative assessment of disease activity in rheumatoid arthritis (RA) is important for patient management, and additional objective information may aid rheumatologists in clinical decision making. We validated a recently developed multibiomarker disease activity (MBDA) test relative to clinical disease activity in diverse RA cohorts. Methods Serum samples were obtained from the Index for Rheumatoid Arthritis Measurement, Brigham and Women's Hospital Rheumatoid Arthritis Sequential Study, and Leiden Early Arthritis Clinic cohorts. Levels of 12 biomarkers were measured and combined according to a prespecified algorithm to generate the composite MBDA score. The relationship of the MBDA score to clinical disease activity was characterized separately in seropositive and seronegative patients using Pearson's correlations and the area under the receiver operating characteristic curve (AUROC) to discriminate between patients with low and moderate/high disease activity. Associations between changes in MBDA score and clinical responses 6–12 weeks after initiation of anti–tumor necrosis factor or methotrexate treatment were evaluated by the AUROC. Results The MBDA score was significantly associated with the Disease Activity Score in 28 joints using the C-reactive protein level (DAS28-CRP) in both seropositive (AUROC 0.77, P < 0.001) and seronegative (AUROC 0.70, P < 0.001) patients. In subgroups based on age, sex, body mass index, and treatment, the MBDA score was associated with the DAS28-CRP (P < 0.05) in all seropositive and most seronegative subgroups. Changes in the MBDA score at 6–12 weeks could discriminate both American College of Rheumatology criteria for 50% improvement responses (P = 0.03) and DAS28-CRP improvement (P = 0.002). Changes in the MBDA score at 2 weeks were also associated with subsequent DAS28-CRP response (P = 0.02). Conclusion Our findings establish the criterion and discriminant validity of a novel multibiomarker test as an objective measure of RA disease activity to aid in the management of RA in patients with this condition.

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TL;DR: The prevalence of extraglandular manifestations in primary Sjögren's syndrome (SS) among participants enrolled in the Sj Ögren's International Collaborative Clinical Alliance (SICCA) Registry is studied.
Abstract: Sjogren’s Syndrome (SS) is one of the most common autoimmune diseases, with an estimated prevalence of approximately 0.6% and a 20:1 female predilection (1, 2). SS may occur in isolation and has been referred to as primary Sjogren’s Syndrome (pSS) or in conjunction with another connective tissue disease, most commonly rheumatoid arthritis (RA) or systemic lupus erythematosus (SLE). This association is often termed secondary Sjogren’s Syndrome (sSS). The cardinal features of SS, including keratoconjunctivitis sicca and salivary gland hypofunction, presumably result from progressive lymphocytic inflammation in affected exocrine glands. While the hallmark features of SS are related to exocrine gland dysfunction, there is substantial evidence that pSS is a systemic autoimmune process. Such evidence includes: 1) the frequent presence of auto-antibodies [e.g., anti-nuclear antibodies (ANA), Sjogren’s Syndrome A (SSA/Ro) and Sjogren’s Syndrome B (SSB/La) antibodies]; 2) the presence of SS in conjunction with other systemic connective tissue diseases; and 3) the reported association of SS with a number of extra-glandular manifestations (EGM). There is an extensive literature describing EGM in SS. One of the earliest reported associations was the increased incidence of lymphoma in patients with SS (3–7). Several reports describe the occurrence of other EGM such as neurologic, pulmonary and other organ specific diseases. For example, renal tubular acidosis, thyroiditis, primary biliary cirrhosis and autoimmune hepatitis are classically thought to be related to SS. However the prevalence of these disorders among SS patients varies widely between cohorts and the association of these EGM with SS is less well defined. In the current study, we describe the prevalence of EGM among participants in the Sjogren’s International Collaborative Clinical Alliance (SICCA) registry (8) and we examine associations between EGM and specific objective phenotypic features of SS. SICCA participants are recruited worldwide into a registry designed to support studies of etiologic factors and outcomes in SS. The registry also provides an opportunity to study EGM prevalence in individuals suspected to have SS, but who fail to meet the 2002 American-European Consensus Group (AECG) criteria for SS. The diversity of the collection in terms of ethnicity, recruitment source, and disease severity, in conjunction with the extensive data collected on each participant, provide a valuable resource for studying EGM in SS.

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TL;DR: To formulate consensus treatment plans (CTPs) for induction therapy of newly diagnosed proliferative lupus nephritis (LN) in juvenile systemic lUPus erythematosus (SLE).
Abstract: Objective To formulate consensus treatment plans (CTPs) for induction therapy of newly-diagnosed proliferative lupus nephritis (LN) in juvenile systemic lupus erythematosus (jSLE).

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TL;DR: To assess the determinants of patients' (PTGL) and physicians' (MDGL) global assessment of rheumatoid arthritis (RA) activity and factors associated with discordance among them, a large number of patients and physicians from around the world are surveyed.
Abstract: Objective. To assess the determinants of patients’ (PTGL) and physicians’ (MDGL) global assessment of rheumatoid arthritis (RA) activity and factors associated with discordance among them. Methods. A total of 7,028 patients in the Quantitative Standard Monitoring of Patients with RA study had PTGL and MDGL assessed at the same clinic visit on a 0 –10-cm visual analog scale (VAS). Three patient groups were defined: concordant rating group (PTGL and MDGL within 2 cm), higher patient rating group (PTGL exceeding MDGL by >2 cm), and lower patient rating group (PTGL less than MDGL by >2 cm). Multivariable regression analysis was used to identify determinants of PTGL and MDGL and their discordance. Results. The mean SD VAS scores for PTGL and MDGL were 4.01 2.70 and 2.91 2.37, respectively. Pain was overwhelmingly the single most important determinant of PTGL, followed by fatigue. In contrast, MDGL was most influenced by swollen joint count (SJC), followed by erythrocyte sedimentation rate (ESR) and tender joint count (TJC). A total of 4,454 (63.4%), 2,106 (30%), and 468 (6.6%) patients were in the concordant, higher, and lower patient rating groups, respectively. Odds of higher patient rating increased with higher pain, fatigue, psychological distress, age, and morning stiffness, and decreased with higher SJC, TJC, and ESR. Lower patient rating odds increased with higher SJC, TJC, and ESR, and decreased with lower fatigue levels. Conclusion. Nearly 36% of patients had discordance in RA activity assessment from their physicians. Sensitivity to the “disease experience” of patients, particularly pain and fatigue, is warranted for effective care of RA.

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TL;DR: The objective was to develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile LS.
Abstract: Objective Juvenile localized scleroderma (LS) is a chronic inflammatory skin disorder associated with substantial morbidity and disability Although a wide range of therapeutic strategies has been reported in the literature, a lack of agreement on treatment specifics and accepted methods for clinical assessment has made it difficult to compare approaches and identify optimal therapy Our objective was to develop standardized treatment plans, clinical assessments, and response criteria for active, moderate to high severity juvenile LS Methods A core group of pediatric rheumatologists, dermatologists, and a lay advisor was engaged by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) to develop standardized treatment plans and assessment parameters for juvenile LS using consensus methods/nominal group techniques Recommendations were validated in 2 face-to-face conferences with a larger group of practitioners with expertise in juvenile LS and with the full membership of CARRA, which encompasses the majority of pediatric rheumatologists in the US and Canada Results Consensus was achieved on standardized treatment plans that reflect the prevailing treatment practices of CARRA members Standardized clinical assessment methods and provisional treatment response criteria were also developed Greater than 90% of pediatric rheumatologists responding to a survey (66% of CARRA membership) affirmed the final recommendations and agreed to utilize these consensus plans to treat patients with juvenile LS Conclusion Using consensus methodology, we have developed standardized treatment plans and assessment methods for juvenile LS The high level of support among pediatric rheumatologists will support future comparative effectiveness studies and enable the development of evidence-based guidelines for the treatment of juvenile LS

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TL;DR: The roles of infertility, pregnancy loss, and personal choice on family size in women with rheumatoid arthritis and systemic lupus erythematosus are determined.
Abstract: Objective Women with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE) have fewer children on average than other women. We sought to determine the roles of infertility, pregnancy loss, and personal choice on family size in women with these diseases. Methods A reproductive history questionnaire was completed by women with RA and SLE participating in a longitudinal observational study. Within each disease cohort, participants were divided into 3 groups: those interested in having children at symptom onset who had either fewer children than planned (group A) or the same number as planned (group B), and those no longer interested in having children at diagnosis (group C). Results Of the 578 RA and 114 SLE women surveyed, >60% were in group C. Of those interested in having children, 55% with RA and 64% with SLE had fewer children than originally planned. Among women with RA, group A had 1 less pregnancy, 1 less live birth, and an infertility rate 1.5 times higher than group B; the miscarriage rate was similar in both groups. Compared to SLE group B, SLE group A had a similar number of pregnancies, but a 3-fold higher rate of miscarriage and 1 less live birth. Concerns about child health and personal welfare were associated with a lower pregnancy rate. Conclusion In this population, more than one-half of young women with RA or SLE had fewer biologic children than desired. While patient choice plays a role, infertility in RA patients and miscarriage in SLE patients are also important.

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TL;DR: To investigate the clinical features of dermatomyositis (DM) and clinically amyopathic DM (CADM) patients with the presence of anti–melanoma differentiation–associated gene 5 (anti–MDA‐5) antibodies, a large number of patients are diagnosed with DM.
Abstract: Objective To investigate the clinical features of dermatomyositis (DM) and clinically amyopathic DM (CADM) patients with the presence of anti–melanoma differentiation–associated gene 5 (anti–MDA-5) antibodies. Methods We screened the serum anti–MDA-5 antibody levels of 140 patients with various connective tissue diseases (CTDs), including 32 with DM and 32 with CADM, or idiopathic pulmonary fibrosis (IPF). The clinical courses of DM/CADM patients with a positive expression of anti–MDA-5 antibodies were delineated. Results Anti–MDA-5 antibodies were detected at a significantly higher frequency in CADM patients than in DM patients (12 of 32 versus 3 of 32; P = 0.016), but were not detected in patients with other CTDs or IPF and healthy controls. Patients with a positive expression of anti–MDA-5 antibodies developed significantly more skin ulcerations (12 of 15 versus 4 of 49; P 500 units/ml were resistant to the treatment and died of respiratory failure in a short period of time. Conclusion Anti–MDA-5 antibody levels closely correlate with the severity of skin ulcerations, ILD, and the prognosis of the disease. Dynamic observation of serum anti–MDA-5 antibody levels would be helpful in predicting the course of ILD and facilitating better therapeutic targeting.

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TL;DR: Whether the prevalence of tibiofemoral joint space narrowing differs by sex and ethnic groups and to what extent such a difference is accounted for by knee malalignment is assessed.
Abstract: Objective We conducted a cross-sectional study to describe the prevalence of tibiofemoral joint space narrowing (JSN) in medial and lateral compartments and assess whether it differs by sex and ethnic groups, and, if it does, to what extent such a difference is accounted for by knee malalignment. Methods The Multicenter Osteoarthritis Study is an observational study of persons ages 50–79 years with either symptomatic knee osteoarthritis or high risk of disease. Knee radiographs were assessed for JSN in each tibiofemoral compartment. Mechanical axis angle was measured using full-extremity films. We compared the proportion of knees with medial compartment JSN and with lateral JSN between men and women, as well as between whites and African Americans, using a logistic regression model adjusting for covariates (race or sex and body mass index, age, education, and clinic site). We used generalized estimating equations to account for correlation between 2 knees within a person. Results Of 5,202 knees (2,652 subjects), 1,532 (29.5%) had medial JSN and 427 (8.2%) had lateral JSN. Lateral JSN was more prevalent in the knees of women than in men (odds ratio [OR] 1.9, 95% confidence interval [95% CI] 1.5–2.4) and was also higher in the knees of African Americans than in whites (OR 2.4, 95% CI 1.7–3.3). Further adjustment for malalignment attenuated the OR for sex but not the OR for race. Conclusion Women and African Americans are more likely to have lateral JSN than men and whites, respectively. Valgus malalignment may contribute to the higher prevalence in women.

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TL;DR: To propose a common nomenclature to refer to individuals who fulfill the American College of Rheumatology classification criteria for systemic lupus erythematosus (SLE) during childhood or adolescence.
Abstract: Objective To propose a common nomenclature to refer to individuals who fulfill the American College of Rheumatology classification criteria for systemic lupus erythematosus (SLE) during childhood or adolescence. Methods The medical literature was reviewed for studies conducted in the target population between 1960 and December 2011 to obtain information about the terms used to refer to such children and adolescents. We reviewed the threshold ages used and disease features considered to discriminate these individuals from patients with onset of SLE during adulthood. Furthermore, the nomenclature used in other chronic diseases with onset during both childhood and adulthood was assessed. Results There was an astonishing variability in the age cutoffs used to define SLE onset prior to adulthood, ranging from 14–21 years, but most studies used age 18 years. The principal synonyms in the medical literature were SLE without reference to the age at onset of disease, childhood-onset SLE, juvenile SLE, and pediatric (or paediatric) SLE. Conclusion Based on the definition of childhood, in analogy with other complex chronic diseases commencing prior to adulthood, and given the current absence of definite genetic variations that discriminate adults from children, the term childhood-onset SLE is proposed when referring to individuals with onset of SLE prior to age 18 years.

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TL;DR: It is described how disease activity, damage, and the accrual of key autoantibodies in an inception systemic lupus erythematosus (SLE) cohort are described.
Abstract: Objective We describe disease activity, damage, and the accrual of key autoantibodies in an inception systemic lupus erythematosus (SLE) cohort. Methods. The Systemic Lupus International Collaborating Clinics (SLICC) International Research Network, comprising 27 centers from 11 countries, has followed an inception cohort of SLE patients yearly according to a standardized protocol. Of these patients, 298 were followed for a minimum of 5 years and constitute the study population. Disease activity was assessed using the SLE Disease Activity Index 2000 (SLEDAI-2K) and damage was assessed using the SLICC/American College of Rheumatology Damage Index (SDI). Antinuclear antibody (ANA), anti-DNA, and anticardiolipin antibody (aCL) levels and lupus anticoagulant were assessed yearly. Descriptive statistics were generated and repeated-measures general linear models were used to evaluate SLEDAI-2K and SDI over time between whites and nonwhites. Results. Of the 298 patients, 87% were women, 55% were white, 12% were African American, 14% were Asian, 16% were Hispanic, and 2% were categorized as "other." At enrollment, the mean age was 35.3 years, the mean SLEDAI-2K score was 5.9, and the mean disease duration was 5.5 months. Mean SLEDAI-2K scores decreased in the first year and then remained low. SLEDAI-2K scores were significantly lower at each year in whites compared to nonwhites. Mean SDI scores increased progressively over 5 years; there was no significant difference between whites and nonwhites. As expected, ANA positivity was high and anti-DNA positivity was relatively low at enrollment, and both increased over 5 years. Although lupus anticoagulant increased slightly over 5 years, aCL positivity did not. Conclusion. Disease activity in newly diagnosed patients decreases over their first 5 years, while damage increases. Antibody positivity ran variable courses over this period.

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TL;DR: To describe the experiences and perspectives of children and adolescents living with juvenile idiopathic arthritis (JIA), a network of peer-to-peer support groups was established to provide real-time information about symptoms and treatment options.
Abstract: Objective To describe the experiences and perspectives of children and adolescents living with juvenile idiopathic arthritis (JIA). Methods We conducted a systematic review of qualitative studies that explored the experiences of children living with JIA. We searched electronic databases (to week 2 of July 2011) and reference lists of relevant articles. Results Twenty-seven studies that reported the experiences of more than 542 participants were included. Six major themes were identified: aversion to being different (unrelenting and unpredictable pain, disablement, internal disfigurement, differential treatment, and forced dependency on others); striving for normality (preserving social identity, resourcefulness, sense of community, focus on remission, and mastery over body and pain); stigma and misunderstanding (trivialization of disease, invisible pain, and discrimination); suspension in uncertainty (control versus powerlessness, hope versus disappointment); managing treatment (benefits of taking medicines, respect and involvement in health care, and motivation for physical therapy); and desire for knowledge (medical treatment and advances, lifestyle management). Conclusion JIA disrupts a child's sense of normality and impairs his or her capacity for social participation. Children with JIA have a sense of being misunderstood and stigmatized, and they feel perpetually caught between having hope and control over their bodies and overwhelming pain and despair. To increase their confidence, the ability to manage pain, and their resourcefulness for self-management, children need ongoing information about treatments and lifestyle management, strong social support, community advocacy, and active involvement in their own health decision making.

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TL;DR: To determine the frequency of incident vertebral fractures (IVF) 12 months after glucocorticoid (GC) initiation in children with rheumatic diseases and to identify children at higher risk.
Abstract: Objective. To determine the frequency of incident vertebral fractures (IVF) 12 months after glucocorticoid (GC) initiation in children with rheumatic diseases and to identify children at higher risk. Methods. Children with rheumatic diseases initiating GC were enrolled in a prospective observational study. Annual spine radiographs were evaluated using the Genant semiquantitative method. Spine areal bone mineral density (aBMD) was measured every 6 months. Clinical features, including cumulative GC dose, back pain, disease and physical activity, calcium and vitamin D intake, and spine aBMD Z scores, were analyzed for association with IVF. Results. Seven (6%) of 118 children (95% confidence interval 2.9 –11.7%) had IVF. Their diagnoses were: juvenile dermatomyositis (n 2), systemic lupus erythematosus (n 3), systemic vasculitis (n 1), and mixed connective tissue disease (n 1). One child was omitted from the analyses after 4 months because of osteoporosis treatment for symptomatic IVF. Children with IVF received on average 50% more GC than those without (P 0.030), had a greater increase in body mass index (BMI) at 6 months (P 0.010), and had greater decrements in spine aBMD Z scores in the first 6 months (P 0.048). Four (67%) of 6 children with IVF and data to 12 months had spine aBMD Z scores less than 2.0 at 12 months compared to 16% of children without IVF (P 0.011). Conclusion. The incidence of VF 12 months following GC initiation was 6%; most children were asymptomatic. Children with IVF received more GC, had greater increases in BMI, and had greater declines in spine aBMD Z scores in the first 6 months.