Showing papers in "Arthritis Care and Research in 2014"

Overall and cause-specific mortality in patients with systemic lupus erythematosus: a meta-analysis of observational studies.

Abstract: Objective To determine the magnitude of risk from all-cause and cause-specific mortality in patients with systemic lupus erythematosus (SLE) compared to the general population through a meta-analysis of observational studies. Methods We searched the Medline and Embase databases from their inception to October 2011. Observational studies that met the following criteria were assessed: 1) a prespecified SLE definition; 2) overall and/or cause-specific deaths, including cardiovascular disease (CVD), infections, malignancy, and renal disease; and 3) reported standardized mortality ratios (SMRs) and 95% confidence intervals (95% CIs). We calculated weighted–pooled summary estimates of SMRs (meta-SMRs) for all-cause and cause-specific mortality using the random-effects model and tested for heterogeneity using the I2 statistic by using Stata/IC statistical software. Results We identified 12 studies comprising 27,123 patients with SLE (4,993 observed deaths) that met the inclusion criteria. Overall, there was a 3-fold increased risk of death in patients with SLE (meta-SMR 2.98, 95% CI 2.32–3.83) when compared with the general population. The risks of death due to CVD (meta-SMR 2.72, 95% CI 1.83–4.04), infection (meta-SMR 4.98, 95% CI 3.92–6.32), and renal disease (SMR 7.90, 95% CI 5.50–11.00) were significantly increased. Mortality due to malignancy was the only cause-specific entity not increased in SLE (meta-SMR 1.19, 95% CI 0.89–1.59). Conclusion The published data indicated a 3-fold increase in all-cause mortality in patients with SLE compared to the general population. Additionally, all cause-specific mortality rates were increased except for malignancy, with renal disease having the highest mortality risk.

Effect of methotrexate, anti-tumor necrosis factor α, and rituximab on the immune response to influenza and pneumococcal vaccines in patients with rheumatoid arthritis: a systematic review and meta-analysis.

Abstract: Objective To assess the current literature on the impact of rheumatoid arthritis (RA) treatments on the humoral response to pneumococcal and influenza vaccines Methods We systematically searched the literature for studies evaluating the immune response to vaccines in RA patients receiving methotrexate (MTX) and/or biologic agents The efficacy of vaccination, assessed by the response rate based on increased antibody titers before and 3–6 weeks after vaccination, was extracted by one investigator and verified by another Results In total, 12 studies were included RA patients mainly received MTX, anti–tumor necrosis factor α (anti-TNFα), or rituximab (RTX) Influenza vaccination response was reduced for RTX (43 patients; pooled odds ratio [OR] 044 [95% confidence interval (95% CI) 017–112] for H1N1, OR 011 [95% CI 004–031] for H3N2, and OR 029 [95% CI 010–081] for B) but not for anti-TNFα (308 patients; OR 093 [95% CI 036–237] for H1N1, OR 079 [95% CI 034–183] for H3N2, and OR 079 [95% CI 037–170] for B) For MTX, results differed depending on the method of analysis (222 patients; OR 035 [95% CI 018–066] for at least 2 strains, ORs were close to 10 in the single strain analysis) Pneumococcal vaccination response was reduced for 139 patients receiving MTX compared with controls (OR 033 [95% CI 020–054] for serotype 6B and OR 058 [95% CI 036–094] for 23F) but not for anti-TNFα (258 patients; OR 096 [95% CI 057–159] for 6B and OR 120 [95% CI 057–254] for 23F) For RTX, the response was reduced (88 patients; OR 025 [95% CI 011–058] for 6B and OR 021 [95% CI 004–105] for 23F) Conclusion MTX decreases humoral response to pneumococcal vaccination and may impair response to influenza vaccination The immune response to both vaccines is reduced with RTX but not with anti-TNFα therapy in RA patients

Defining enthesitis in spondyloarthritis by ultrasound: results of a Delphi process and of a reliability reading exercise.

Abstract: Objective: To standardize ultrasound (US) in enthesitis. Methods: An initial Delphi exercise was undertaken to define US-detected enthesitis and its core components. These definitions were subsequently tested on static images taken from spondyloarthritis patients in order to evaluate their reliability. Results: Excellent agreement (>80%) was obtained for including hypoechogenicity, increased thickness of the tendon insertion, calcifications, enthesophytes, erosions, and Doppler activity as core elementary lesions of US-detected enthesitis. US definitions were subsequently obtained for each elementary component. On static images, the intraobserver reliability showed a high degree of variability for the detection of elementary lesions, with kappa coefficients ranging from 0.13-1. The interobserver kappa values were variable, with the lowest kappa coefficient for enthesophytes (0.24) and the highest coefficient for Doppler activity at the enthesis (0.63). Conclusion: This is the first consensus-based US definition of enthesitis and its elementary components and the first step performed to ensure a higher degree of homogeneity and comparability of results between studies and in daily clinical work.

Usefulness of 2‐[18F]‐fluoro‐2‐deoxy‐d‐glucose–Positron Emission Tomography/Computed Tomography for Staging and Evaluation of Treatment Response in IgG4‐Related Disease: A Retrospective Multicenter Study

Abstract: Objective To evaluate the usefulness of 2-[18F]-fluoro-2-deoxy-d-glucose–positron emission tomography/computed tomography (FDG-PET/CT) in IgG4-related disease (IgG4-RD) for the staging of the disease and the followup under treatment. Methods All patients included in the French IgG4-RD registry who underwent ≥1 FDG-PET/CT scan were included in the study. Clinical, biologic, pathologic, radiologic, and FDG-PET/CT qualitative and quantitative findings were retrospectively collected and analyzed. Results Twenty-one patients were included in the study and 46 FDG-PET/CT examinations were evaluated. At either diagnosis or relapse, all evaluated patients presented abnormal 18F-FDG uptake in typical IgG4-RD localizations. In most cases, FDG-PET/CT was more sensitive than conventional imaging to detect organ involvement, especially in arteries, salivary glands, and lymph nodes. In few cases (small-sized lesions and brain or kidney contiguous lesions), false-negative results were noted. Evaluation before and after treatment showed in most cases a good correlation of FDG-PET/CT results with treatment response and disease activity. Conclusion This large retrospective study shows that FDG-PET/CT imaging is useful for the staging of IgG4-RD. Moreover, FDG-PET/CT is useful to assess the response to treatment during followup.

Primary Sjögren's syndrome: diagnostic and prognostic value of salivary gland ultrasonography using a simplified scoring system.

Abstract: Objective: To determine the usefulness and prognostic value of a simplified salivary gland ultrasonography (SGUS) scoring system in primary Sjogren's syndrome (pSS). Methods: Patients with pSS (n=105) and controls (n=57) were evaluated using a simplified SGUS scoring system. Parenchymal homogeneity in salivary glands was graded from 0 to 3, grades 0 (normal) and 1 (mild inhomogeneity) being interpreted as normal or unspecific, and grades 2 (several rounded) and 3 (numerous or confluent hypoechoic lesions) as pSS-typical. Associations between SGUS and clinical, histological and laboratory disease characteristics were analyzed. Results: The characteristic hypoechoic lesions (score 2 or 3) were found in 52% of pSS patients and in 1 (1.8%) of controls, p<0.001. Specificity and positive predictive value of abnormal SGUS for pSS were both 98%, sensitivity and negative predictive value were 52 and 53%. Age or disease duration did not influence the SGUS result. Dryness did not differ between normal or abnormal SGUS. However, patients with pathological SGUS had significantly more often signs and symptoms of systemic complications, higher disease activity and more frequently markers of lymphoma development, such as salivary gland swelling, skin vasculitis, germinal center-like structures in salivary gland biopsy and CD4(+) T-lymphocytopenia. Conclusion: SGUS using a simplified score for assessment of parenchyma dyshomogeneity is highly specific for pSS and offers the advantage of identifying patients with severe disease or at lymphoma risk. Early disease may however be missed. It is easy and rapidly performed and may be considered as an item in future modified classification criteria. © 2013 American College of Rheumatology. (Less)

Medication adherence in gout: a systematic review

Abstract: Objective Recent data suggesting the growing problem of medication nonadherence in gout have called for the need to synthesize the burden, determinants, and impacts of the problem. Our objective was to conduct a systematic review of the literature examining medication adherence among patients with gout in real-world settings. Methods We conducted a search of Medline, Embase, International Pharmaceutical Abstracts, PsycINFO, and CINAHL databases and selected studies of gout patients and medication adherence in real-world settings. We extracted information on study design, sample size, length of followup, data source (e.g., prescription records versus electronic monitoring versus self-report), type of nonadherence problem evaluated, adherence measures and reported estimates, and determinants of adherence reported in multivariable analyses. Results We included 16 studies that we categorized according to methods used to measure adherence, including electronic prescription records (n = 10), clinical records (n = 1), electronic monitoring devices (n = 1), and self-report (n = 4). The burden of nonadherence was reported in all studies, and among studies based on electronic prescription records, adherence rates were all below 0.80 and the proportion of adherent patients ranged from 10–46%. Six studies reported on determinants, with older age and having comorbid hypertension consistently shown to be positively associated with better adherence. One study showed the impact of adherence on achieving a serum uric acid target. Conclusion With less than half of gout patients in real-world settings adherent to their treatment, this systematic review highlights the importance of health care professionals discussing adherence to medications during encounters with patients.

Expert consensus on best practices for post-acute rehabilitation after total hip and knee arthroplasty: a Canada and United States Delphi study.

Abstract: Objective To synthesize professional and patient expertise with available evidence to recommend best practices for post–acute rehabilitation following primary total hip arthroplasty (THA) and total knee arthroplasty (TKA) for osteoarthritis (OA). Methods Two expert panels of clinicians, researchers, and patients from Canada and the US participated in a 3-round, online Delphi survey. Results Consensus was reached on 22 THA and 24 TKA best practice key statements. Recommendations common to both procedures included the need for supervised rehabilitation interventions provided by trained health professionals early after discharge from the acute care setting to optimize patient outcomes. Personal and environmental contextual factors were identified as influencing the process and outcomes of THA and TKA rehabilitation. Routine outcome assessment was recommended and several standardized outcome tools identified. Short-term followup care in the first 2 years postsurgery was recommended for both procedures. Specifics on timing, rehabilitation providers, need for long-term followup, and interventions differed for THA and TKA. Some recommendations received different levels of support based on the type of panelist (patient, physical therapist, surgeon), professional role (clinician, researcher), and/or country. Conclusion A rigorous consensus method led to key recommendations for post–acute rehabilitation after primary THA and TKA for OA, which together with available evidence and acknowledgment of contextual factors will inform the development of clinical practice guidelines. This is an important step toward reducing practice variation, closing the evidence–practice gap, and improving the quality of rehabilitation services after THA and TKA.

Survival and Predictors of Mortality in Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension: Outcomes From the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry

Abstract: Objective To assess cumulative survival rates and identify independent predictors of mortality in patients with incident systemic sclerosis (SSc)–associated pulmonary arterial hypertension (PAH) who had undergone routine screening for PAH at SSc centers in the US. Methods The Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma registry is a prospective registry of SSc patients at high risk for PAH or with definite pulmonary hypertension diagnosed by right-sided heart catheterization within 6 months of enrollment. Only patients with World Health Organization group I PAH (mean pulmonary artery pressure ≥25 mm Hg and pulmonary capillary wedge pressure ≤15 mm Hg without significant interstitial lung disease) were included in these analyses. Results In total, 131 SSc patients with incident PAH were followed for a mean ± SD of 2.0 ± 1.4 years. The 1-, 2-, and 3-year cumulative survival rates were 93%, 88%, and 75%, respectively. On multivariate analysis, age >60 years (hazard ratio [HR] 3.0, 95% confidence interval [95% CI] 1.1–8.4), male sex (HR 3.9, 95% CI 1.1–13.9), functional class (FC) IV status (HR 6.5, 95% CI 1.8–22.8), and diffusing capacity for carbon monoxide (DLco) <39% predicted (HR 4.2, 95% CI 1.3–13.8) were significant predictors of mortality. Conclusion This is the largest study describing survival in patients with incident SSc-associated PAH followed up at multiple SSc centers in the US who had undergone routine screening for PAH. The survival rates were better than those reported in other recently described SSc-associated PAH cohorts. Severely reduced DLco and FC IV status at the time of PAH diagnosis portended a poor prognosis in these patients.

Mortality Trends in Patients With Early Rheumatoid Arthritis Over 20 Years: Results From the Norfolk Arthritis Register

Abstract: Objective To examine mortality rates in UK patients with early rheumatoid arthritis (RA) from 1990–2011 and compare with population trends. Methods The Norfolk Arthritis Register (NOAR) recruited adults with ≥2 swollen joints for ≥4 weeks: cohort 1 (1990–1994), cohort 2 (1995–1999), and cohort 3 (2000–2004). At baseline, serum rheumatoid factor and anti–citrullinated protein antibody were measured and the 2010 American College of Rheumatology/European League Against Rheumatism RA classification criteria were applied. Patients were followed for 7 years, until emigration or death. The UK Office for National Statistics notified the NOAR of the date and cause of deaths, and provided mortality rates for the Norfolk population. All-cause and cardiovascular-specific standardized mortality ratios (SMRs) were calculated. Poisson regression was used to compare mortality rate ratios (MRRs) between cohorts and then, with cubic splines, to model rates by calendar year. Analyses were performed in patients 1) with early inflammatory arthritis, 2) classified as having RA, and 3) autoantibody positive. Results A total of 2,517 patients were included, with 1,639 women (65%) and median age 55 years, and 1,419 (56%) fulfilled the 2010 RA criteria. All-cause and cardiovascular-specific SMRs were significantly elevated in the antibody-positive groups. There was no change in mortality rates over time after accounting for changes in the population rates. In RA patients, all-cause MRRs, compared to cohort 1, were 1.13 (95% confidence interval [95% CI] 0.84–1.52) and 1.00 (95% CI 0.70–1.43) in cohorts 2 and 3, respectively. Conclusion Mortality rates were increased in patients with RA and SMRs were particularly elevated in those who were autoantibody positive. Compared to the general population, mortality rates have not improved over the past 20 years.

Trends in prescription of opioids from 2003-2009 in persons with knee osteoarthritis.

Abstract: Symptomatic knee osteoarthritis (OA) affects more than 9.3 million US adults and is a leading cause of disability (1). Increased age and obesity strongly correlate with greater risk of developing OA (2, 3). Because there are no disease modifying regimens currently available, treatments for knee OA focus on symptom relief and functional restoration, and include: exercise, weight reduction, over-the-counter drugs (OTCs), injections, assistive devices, prescription NSAIDS, opioids and possibly total knee replacement (TKR) (4). Persons with symptomatic knee OA live on average 26 years from OA diagnosis, highlighting the importance of long term pain management (5). The choices for effective pain management are guided by patient characteristics and treatment guidelines. The American Academy of Orthopedic Surgeons (AAOS), the American College of Rheumatology (ACR), and the European League Against Rheumatism (EULAR), do not provide strong recommendations for or against the use of opioids in the treatment of OA, while other organizations, including the Osteoarthritis Research Society International (OARSI), the American Geriatrics Society (AGS), and the American Pain Society (APS), give limited guidance on how and when opioids might be best used (6–11). A growing body of research has raised awareness of the adverse effects of non-steroidal anti-inflammatory drug (NSAIDs) use, especially among persons with multiple comorbidities. Concerns regarding the increased risk of cardiac adverse events with COX-2 inhibitors led to warnings and, in 2004, the voluntary withdrawal from the market of rofecoxib (Vioxx), a commonly prescribed COX-2 inhibitor (12–14). As questions surrounding the adverse effects of NSAIDs and COX-2 inhibitors for the treatment of persistent pain related to osteoarthritis have multiplied in the past several years, the use of opiates has emerged as a treatment option that may provide effective pain relief with less risk than NSAIDs. Even as opiates have emerged as reasonable agents for select patients with symptomatic OA who are unresponsive to NSAIDs or at high risk of NSAID toxicity, several trends have highlighted the potential harms of increasing opiate use, at the individual and societal levels. A growing body of controlled trials has documented that opiate use is associated with a range of toxicities including nausea, vomiting, constipation and somnolence, among others, while additional research documents associations between opiate use and fractures, cardiovascular events and death (15–17). In addition, prescription opiates are now the leading source of illicit drug use in the US, costing over 50 billion dollars annually in lost productivity, criminal justice costs, drug abuse treatment and medical complications (18–20). Thus trends in opiate use are relevant both to the challenge of managing chronic pain and to the growing national policy problem of illicit use of prescription opiates. In view of these pressing clinical and policy problems, we sought to examine the temporal trends in opioid prescriptions in elderly persons with knee OA using national survey of Medicare beneficiaries and to identify factors associated with greater opioids prescription in this population.

Subcutaneous Tocilizumab Versus Placebo in Combination With Disease-Modifying Antirheumatic Drugs in Patients With Rheumatoid Arthritis

Abstract: Objective The efficacy and safety of subcutaneous tocilizumab (TCZ-SC) versus subcutaneous placebo (PBO-SC) was evaluated in patients with rheumatoid arthritis who had an inadequate response to disease-modifying antirheumatic drugs in the BREVACTA study.

Management of osteoarthritis in general practice in Australia.

Abstract: OBJECTIVE: To describe management of osteoarthritis (OA) of the hip (OA-hip) and knee (OA-knee) by Australian general practitioners (GPs). METHODS: We analyzed data from the Bettering the Evaluation and Care of Health program, from April 1, 2005 to March 31, 2010. Patient and GP characteristics and encounter management data were extracted. Data were classified by the International Classification of Primary Care, version 2, and summarized using descriptive statistics and 95% confidence intervals around point estimates. RESULTS: There were 489,900 GP encounters at which OA was managed (rate of 26.4 per 1,000 encounters). OA-hip was managed at a rate of 2.3 per 1,000 encounters (n = 1,106, 8.6% OA) and OA-knee at a rate of 6.2 per 1,000 (n = 3,058, 23.7% OA). The encounter management rate per 1,000 for OA-hip was higher among non-metropolitan dwellers (2.85 per 1,000 versus 1.97 per 1,000) and lower for non-English-speaking people (1.53 per 1,000 encounters versus 2.39 per 1,000). The rate for OA-knee was higher for non-English-speaking background (8.50 per 1,000 encounters versus 6.24 per 1,000) and lower among indigenous people (3.16 per 1,000 encounters versus 6.46 per 1,000). Referral to an orthopedic surgeon was the most frequently used nonpharmacologic management (OA-knee 17.4 per 100 contacts and OA-hip 17.7 per 100), followed by advice, education, and counselling. As first-line treatment, medication prescription rates (OA-knee 78.7 per 100 contacts and OA-hip 73.2 per 100) were substantially higher than rates of lifestyle management (OA-knee 20.7 per 100 contacts and OA-hip 14.8 per 100). CONCLUSION: OA-hip and OA-knee encounters and management differ. Nonpharmacologic treatments as first-line management were low compared with pharmacologic management rates, and surgical referral rates were high. However, lack of longitudinal data limits definitive assessment of appropriateness of care.

Phase III Study of the Efficacy and Safety of Subcutaneous Versus Intravenous Tocilizumab Monotherapy in Patients With Rheumatoid Arthritis

Abstract: Objective To evaluate the efficacious noninferiority of subcutaneous tocilizumab injection (TCZ-SC) monotherapy to intravenous TCZ infusion (TCZ-IV) monotherapy in Japanese patients with rheumatoid arthritis (RA) with an inadequate response to synthetic and/or biologic disease-modifying antirheumatic drugs (DMARDs). Methods This study had a double-blind, parallel-group, double-dummy, comparative phase III design. Patients were randomized to receive TCZ-SC 162 mg every 2 weeks or TCZ-IV 8 mg/kg every 4 weeks; no DMARDs were allowed during the study. The primary end point was to evaluate the noninferiority of TCZ-SC to TCZ-IV regarding the American College of Rheumatology criteria for 20% improvement in disease activity (ACR20) response rates at week 24 using an 18% noninferiority margin. Additional efficacy, safety, pharmacokinetic, and immunogenicity parameters were assessed. Results At week 24, ACR20 response was achieved in 79.2% (95% confidence interval [95% CI] 72.9, 85.5) of the TCZ-SC group and in 88.5% (95% CI 83.4, 93.5) of the TCZ-IV group; the weighted difference was −9.4% (95% CI −17.6, −1.2), confirming the noninferiority of TCZ-SC to TCZ-IV. Remission rates of the Disease Activity Score in 28 joints using the erythrocyte sedimentation rate and the Clinical Disease Activity Index at week 24 were 49.7% and 16.4% in the TCZ-SC group and 62.2% and 23.1% in the TCZ-IV group, respectively. Serum trough TCZ concentrations were similar between the groups over time. Incidences of all adverse events and serious adverse events were 89.0% and 7.5% in the TCZ-SC group and 90.8% and 5.8% in the TCZ-IV group, respectively. Anti-TCZ antibodies were detected in 3.5% of the TCZ-SC group; no serious hypersensitivity was reported in these patients. Conclusion TCZ-SC monotherapy demonstrated comparable efficacy and safety to TCZ-IV monotherapy. TCZ-SC could provide additional treatment options for patients with RA.

Higher Rates and Clustering of Abnormal Lipids, Obesity, and Diabetes Mellitus in Psoriatic Arthritis Compared With Rheumatoid Arthritis

Abstract: Objective We compared the prevalence and the clustering of the metabolic syndrome (MetS) components (obese body mass index [BMI; ≥30 kg/m2], hypertriglyceridemia, low high-density lipids, hypertension, and diabetes mellitus) in patients with psoriatic arthritis (PsA) and rheumatoid arthritis (RA) in the Consortium of Rheumatology Researchers of North America (CORRONA) Registry. Methods We included CORRONA participants with a rheumatologist-confirmed clinical diagnosis of PsA or RA with complete data. We used a modified definition of MetS that did not include insulin resistance, waist circumference, or blood pressure measurements. Logistic regression models were adjusted for age, sex, and race. Results In the overall CORRONA population, the rates of diabetes mellitus and obesity were significantly higher in PsA compared with RA. In 294 PsA and 1,162 RA participants who had lipids measured, the overall prevalence of MetS in PsA versus RA was 27% versus 19%. The odds ratio (OR) of MetS in PsA versus RA was 1.44 (95% confidence interval [95% CI] 1.05–1.96, P = 0.02). The prevalence of hypertriglyceridemia was higher in PsA compared with RA (38% versus 28%; OR 1.51 [95% CI 1.15–1.98], P = 0.003). The prevalence of type 2 diabetes mellitus was also higher in PsA compared with RA (15% versus 11%; OR 1.56 [95% CI 1.07–2.28], P = 0.02) in the adjusted model. Similarly, higher rates of hypertriglyceridemia and diabetes mellitus were observed in the subgroup of PsA and RA patients with obese BMI. Conclusion Compared with RA, PsA is associated with higher rates of obesity, diabetes mellitus, and hypertriglyceridemia.

Diagnostic Value of Color Doppler Ultrasonography of Temporal Arteries and Large Vessels in Giant Cell Arteritis: A Consecutive Case Series

Abstract: Objective Color Doppler ultrasonography (CDUS) can detect inflammation in the vessel wall. No studies have evaluated the examination of the common carotid artery by CDUS in the diagnostics of giant cell arteritis (GCA). Our aim was to evaluate the combination of CDUS examination of the temporal, axillary, and common carotid arteries in the diagnosis of GCA. Methods Patients ages ≥50 years who were referred to our department between April 2010 and October 2012 and suspected to have GCA were consecutively examined. A positive clinical evaluation for GCA 6 months after the first evaluation by 3 rheumatologists was considered as the gold diagnostic standard. All patients underwent CDUS of the temporal, axillary, and common carotid arteries. A biopsy of the temporal artery was performed for most patients. Results A total of 88 patients were assessed. Forty-six patients were diagnosed to have GCA by the defined gold standard. Forty-eight patients had a positive CDUS of the temporal artery. Forty-six patients diagnosed with GCA had a positive CDUS of the temporal, common carotid, and axillary arteries (100% sensitivity) and 4 patients had a positive CDUS without having GCA (91% specificity). Among the 39 GCA patients that underwent a biopsy, vasculitis was observed in 26 patients (66%), yielding a sensitivity of 67% and a specificity of 95%. Conclusion CDUS of the common carotid, axillary, and temporal arteries had an excellent sensitivity and high specificity to diagnose GCA. CDUS has the potential to replace biopsy in ordinary clinical care without compromising on sensitivity and specificity.

Epidemiology of Primary Sjögren's Syndrome in a French Multiracial/Multiethnic Area

Abstract: Objective To describe the epidemiology of primary Sjogren's syndrome (SS) in a multiracial/multiethnic population. Methods A cross-sectional study with 5 case-retrieval sources identified adults with primary SS living in the Greater Paris area (population 1,172,482 adults) in 2007. Diagnoses were verified by the American–European Consensus Group (AECG) criteria and study-specific enlarged criteria based on the presence of ≥3 of 4 AECG items among subjective oral or ocular dryness, anti-SSA/SSB positivity, and positive minor salivary gland biopsy results. Prevalence estimates were standardized to those for the world population and a 5-source capture–recapture analysis (CRA) was used. Racial/ethnic differences in primary SS features were evaluated. Results In all, 133 subjects met the AECG criteria and 203 met the enlarged criteria. The 2007 prevalence of primary SS was 1.02 cases per 10,000 adults (95% confidence interval [95% CI] 0.85–1.22) for the AECG criteria and 1.52 cases per 10,000 adults (95% CI 1.30–1.76) for the enlarged criteria. The CRA indicated completeness of case findings of ∼90%. Compared to subjects with European backgrounds, those with non-European backgrounds had 2.1–2.3 times higher primary SS prevalence and were younger (P < 0.0001) and were more likely to have polyclonal hypergammaglobulinemia (P < 0.0001) and anti-SSA/SSB antibodies (P = 0.0005 and P < 0.0001 for the AECG and enlarged criteria, respectively). Conclusion The figure of 1.02–1.52 cases per 10,000 adults we found and estimates from the few other population-based census surveys support that the prevalence of diagnosed primary SS is between 1 and 9 cases per 10,000 people (0.01–0.9%) in the general population. Non-European race/ethnicity may be associated with increased primary SS risk and a distinct disease profile.

Defining criteria for disease activity states in nonsystemic juvenile idiopathic arthritis based on a three-variable juvenile arthritis disease activity score.

Abstract: Objective To determine cutoff values for defining the states of inactive disease (ID), low disease activity (LDA; or minimal disease activity), moderate disease activity (MDA), and high disease activity (HDA) using the clinical (3-variable) Juvenile Arthritis Disease Activity Score (cJADAS). Methods For selection of cutoffs, data from a clinical database including 609 children with juvenile idiopathic arthritis (JIA) were used. Optimal cutoffs were determined against external criteria by calculating the 75th and 90th percentile (for ID and LDA) and 10th and 25th percentile (for HDA) of cumulative score distribution and through receiver operating characteristic curve analysis. External criteria included definitions for ID and LDA cutoffs and therapeutic decisions for HDA cutoffs. MDA cutoffs were set at the score interval in-between LDA and HDA cutoffs. Crossvalidation was performed using 2 JIA patient samples (n = 485) and was based on assessment of construct and discriminant validity. Results The selected cutoffs were as follows: ≤1 for ID in both oligoarthritis and polyarthritis; ≤1.5 and ≤2.5 for LDA in oligoarthritis and polyarthritis, respectively; 1.51–4 and 2.51–8.5 for MDA in oligoarthritis and polyarthritis, respectively; and >4 and >8.5 for HDA in oligoarthritis and polyarthritis, respectively. In crossvalidation analyses, the cutoffs showed a strong ability to discriminate between disease activity states defined subjectively by physicians and parents, levels of pain, and presence/absence of functional impairment and disease damage. Conclusion Cutoff values for classifying various disease states in nonsystemic JIA using the cJADAS were developed. The cutoffs revealed good measurement characteristics in crossvalidation analyses and are suited for application in clinical practice and research.

Childhood Arthritis and Rheumatology Research Alliance consensus treatment plans for new-onset polyarticular juvenile idiopathic arthritis.

Abstract: Objective There is no standardized approach to the initial treatment of polyarticular juvenile idiopathic arthritis (JIA) among pediatric rheumatologists. Understanding the comparative effectiveness of the diverse therapeutic options available will result in better health outcomes for polyarticular JIA. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed consensus treatment plans (CTPs) for use in clinical practice to facilitate such studies. Methods A case-based survey was administered to CARRA members to identify the common treatment approaches for new-onset polyarticular JIA. Two face-to-face consensus conferences employed modified nominal group technique to identify treatment strategies, operational case definition, end points, and data elements to be collected. A core workgroup reviewed the relevant literature, refined plans, and developed medication dosing and monitoring recommendations. Results The initial case-based survey identified significant variability among treatment approaches for new-onset polyarticular JIA. We developed 3 CTPs based on treatment strategies for the first 12 months of therapy, as well as case definitions and clinical and laboratory monitoring schedules. The CTPs include a step-up plan (nonbiologic disease-modifying antirheumatic drug [DMARD] followed by a biologic DMARD), an early combination plan (nonbiologic and biologic DMARD combined within a month of treatment initiation), and a biologic only plan. This approach was approved by 96% of the CARRA JIA Research Committee members attending the 2013 CARRA face-to-face meeting. Conclusion Three standardized CTPs were developed for new-onset polyarticular JIA. Coupled with data collection at defined intervals, use of these CTPs will enable the study of their comparative effectiveness in an observational setting to optimize initial management of polyarticular JIA.

Daily walking and the risk of incident functional limitation in knee osteoarthritis: an observational study.

Abstract: Objective Physical activity is recommended to mitigate functional limitations associated with knee osteoarthritis (OA). However, it is unclear whether walking on its own protects against the development of functional limitation. Methods Walking over 7 days was objectively measured as steps/day within a cohort of people with or at risk of knee OA from the Multicenter Osteoarthritis Study. Incident functional limitation over 2 years was defined by performance-based (gait speed 28 of 68) measures. We evaluated the association of steps/day at baseline with developing functional limitation 2 years later by calculating risk ratios adjusted for potential confounders. The number of steps/day that best distinguished risk for developing functional limitation was estimated from the maximum distance from chance on receiver operating characteristic curves. Results Among 1,788 participants (mean age 67 years, mean body mass index 31 kg/m(2) , 60% women), each additional 1,000 steps/day was associated with a 16% and 18% reduction in incident functional limitation by performance-based and self-report measures, respectively. Walking Conclusion More walking was associated with less risk of functional limitation over 2 years. Walking >6,000 steps/day provides a preliminary estimate of the level of walking activity to protect against developing functional limitation in people with or at risk of knee OA.

Prediction of relapse after discontinuation of biologic agents by ultrasonographic assessment in patients with rheumatoid arthritis in clinical remission: high predictive values of total gray-scale and power Doppler scores that represent residual synovial inflammation before discontinuation.

Abstract: Objective This prospective study aimed to determine whether the comprehensive ultrasonographic assessment of synovial inflammation predicts relapse after discontinuation of treatment with a biologic agent in patients with rheumatoid arthritis (RA) in clinical remission. Methods RA patients in clinical remission (Disease Activity Score in 28 joints [DAS28] <2.6) receiving treatment with a biologic agent who agreed to discontinue the treatment were recruited. Patients underwent a comprehensive ultrasound scan on 134 synovial sites in 40 joints and were prospectively followed up for 6 months. Physicians who evaluated the patients during the study period were blinded to the baseline ultrasound findings. Results Forty-two patients receiving either a tumor necrosis factor antagonist or tocilizumab were enrolled. Using the optimal cutoff values determined by receiver operating characteristic curve analysis, relapse rates were significantly higher in patients whose total ultrasound scores at discontinuation were high than in those whose total ultrasound scores were low (P < 0.001 for both total gray-scale and power Doppler scores), whereas the difference between high and low DAS28 was not statistically significant (P = 0.158 by log rank test). Positive and negative predictive values were 80.0% and 73.3% for the total gray-scale score and 88.9% and 74.2% for the total power Doppler score, respectively. Conclusion In RA patients in clinical remission receiving treatment with a biologic agent, residual synovial inflammation determined by comprehensive ultrasound assessment predicted relapse within a short term after discontinuation of the treatment. Our data provide a rationale and groundwork to conduct a large-scale study for establishment of ultrasound-based strategies to optimize the period of treatment with a biologic agent.

Criteria for the Diagnosis of Fibromyalgia: Validation of the Modified 2010 Preliminary American College of Rheumatology Criteria and the Development of Alternative Criteria

Abstract: Objective To validate the 2011 modification of the 2010 American College of Rheumatology (ACR) preliminary criteria for the diagnosis of fibromyalgia (2011ModCr) and develop alternative criteria in a sample of patients with diverse pain disorders that are commonly seen in everyday practice by pain specialists, rheumatologists, and psychologists. Methods Eight clinicians from geographically varied locations in the US evaluated patients with chronic pain and psychiatric disorders using a standard set of questions that included the 2011ModCr questions, the Symptom Impact Questionnaire (SIQR), a 28-area pain location inventory (PLI), and the Short Form 36. Alternative diagnostic criteria were developed from the same data set using logistic regression and receiver operating curve analysis. Results Complete data on 321 patients were evaluated; there were 135 patients with fibromyalgia (according to the 1990 ACR criteria) and 186 patients with 16 other common chronic pain problems. Comparing the 2011ModCr with the 1990 ACR criteria provided a sensitivity of 83%, a specificity of 67%, and a correct classification of 74%. Alternative criteria were derived from the 10-item symptom score from the SIQR symptoms and the 28-area PLI. Maximal diagnostic accuracy was obtained with ≥17 pain sites (range 0–28) and an SIQR symptom score of ≥21 (range 0–50). These alternative criteria had a diagnostic sensitivity of 81%, a specificity of 80%, and a correct classification of 80%. Conclusion The 2011ModCr had robust operating characteristics. Alternative criteria based on symptom items from the SIQR and pain locations from the PLI had comparable operating characteristics, with somewhat better specificity and ease of use.

Association of Exercise Therapy and Reduction of Pain Sensitivity in Patients With Knee Osteoarthritis: A Randomized Controlled Trial

Abstract: Objective Exercise has beneficial effects on pain in knee osteoarthritis (OA), yet the underlying mechanisms are unclear. The purpose of this study was to investigate the effects of exercise on pressure–pain sensitivity in patients with knee OA. Methods In a randomized controlled trial, participants were assigned to 12 weeks of supervised exercise therapy (ET; 36 sessions) or a no attention control group (CG). Pressure–pain sensitivity was assessed by cuff pressure algometry on the calf of the most symptomatic leg. The coprimary outcomes were pressure–pain thresholds (PPTs) and cumulated visual analog scale pain scores during constant pressure for 6 minutes at 125% of the PPT as a measure of temporal summation (TS) of pressure–pain. Secondary outcomes included self-reported pain using the Knee Injury and Osteoarthritis Outcome Score (KOOS) questionnaire. Analyses were based on the “per-protocol” population (participants following the protocol). Results Sixty participants were randomized (31 in ET group, 29 in CG), and the per-protocol population included 48 participants (25 in ET group, 23 in CG). At followup, mean group differences in the change from baseline were 3.1 kPa (95% confidence interval [95% CI] 0.2, 6.0; P = 0.038) for the PPT, 2,608 mm × seconds (95% CI 458, 4,758; P = 0.019) for TS, and 6.8 points (95% CI 1.2, 12.4; P = 0.018) for KOOS pain, all in favor of ET. Conclusion Pressure–pain sensitivity, TS, and self-reported pain are reduced among patients completing a 12-week supervised exercise program compared to a no attention CG. These results demonstrate beneficial effects of exercise on basic pain mechanisms and further exploration may provide a basis for optimized treatment.

Risk of Hospitalized Bacterial Infections Associated With Biologic Treatment Among US Veterans With Rheumatoid Arthritis

Abstract: Objective The comparative risk of infection associated with non–anti–tumor necrosis factor (anti-TNF) biologic agents is not well established. Our objective was to compare risk for hospitalized infections between anti-TNF and non–anti-TNF biologic agents in US veterans with rheumatoid arthritis (RA). Methods Using 1998–2011 data from the US Veterans Health Administration, we studied RA patients initiating rituximab, abatacept, or anti-TNF therapy. Exposure was based upon days supplied (injections) or usual dosing intervals (infusions). Treatment episodes were defined as new biologic agent use. Hazard ratios (HRs) and 95% confidence intervals (95% CIs) for hospitalization for a bacterial infection were estimated from Cox proportional hazards models, adjusting for potential confounders. Results Among 3,152 unique RA patients contributing 4,158 biologic treatment episodes to rituximab (n = 596), abatacept (n = 451), and anti-TNF agents (n = 3,111), the patient mean age was 60 years and 87% were male. The most common infections were pneumonia (37%), skin/soft tissue (22%), urinary tract (9%), and bacteremia/sepsis (7%). Hospitalized infection rates per 100 person-years were 4.4 (95% CI 3.1–6.4) for rituximab, 2.8 (95% CI 1.7–4.7) for abatacept, and 3.0 (95% CI 2.5–3.5) for anti-TNF. Compared to etanercept, the adjusted rate of hospitalized infection was not different for adalimumab (HR 1.4, 95% CI 0.9–2.2), abatacept (HR 1.1, 95% CI 0.6–2.1), or rituximab (HR 1.4, 0.8–2.6), although it was increased for infliximab (HR 2.3, 95% CI 1.3–4.0). Infection risk was greater for those taking prednisone >7.5 mg/day (HR 1.8, 95% CI 1.3–2.7) and in the highest quartile of C-reactive protein (HR 2.3, 95% CI 1.4–3.8) and erythrocyte sedimentation rate (HR 4.1, 95% CI 2.3–7.2) compared to the lowest quartile. Conclusion In older, predominantly male US veterans with RA, the risk of hospitalized bacterial infections associated with rituximab or abatacept was similar to etanercept.

Current evidence of anti-tumor necrosis factor α treatment efficacy in childhood chronic uveitis: a systematic review and meta-analysis approach of individual drugs.

Abstract: Objective To summarize evidence regarding the effectiveness of anti–tumor necrosis factor α (anti-TNFα) treatments in childhood autoimmune chronic uveitis (ACU), refractory to previous disease-modifying antirheumatic drugs (DMARDs). Methods A systematic search between January 2000 and October 2012 was conducted using EMBase, Ovid Medline, Evidence-Based Medicine (EBM) Reviews: American College of Physicians Journal Club, Cochrane libraries, and EBM Reviews. Studies investigating the efficacy of anti-TNFα therapy, in children ages ≤16 years, as the first treatment with a biologic agent for ACU, refractory to topical and/or systemic steroid therapy and at least 1 DMARD, were eligible for inclusion. The primary outcome measure was the improvement of intraocular inflammation, as defined by the Standardization of Uveitis Nomenclature Working Group criteria. We determined a combined estimate of the proportion of children responding to anti-TNFα treatment, including etanercept (ETA), infliximab (INF), or adalimumab (ADA). Results We initially identified 989 articles, of which 148 were potentially eligible. In total, 22 retrospective chart reviews and 1 randomized clinical trial were deemed eligible, thus including 229 children (ADA: n = 31, ETA: n = 54, and INF: n = 144). On pooled analysis of observational studies, the proportion of responding children was 87% (95% confidence interval [95% CI] 75–98%) for ADA, 72% (95% CI 64–79%) for INF, and 33% (95% CI 19–47%) for ETA. There was no difference in the proportion of responders between ADA and INF (χ2 = 3.06, P = 0.08), although both showed superior efficacy compared with ETA (ADA versus ETA: χ2 = 20.9, P < 0.001 and INF versus ETA: χ2 = 20.9, P < 0.001). Conclusion Although randomized controlled trials are needed, the available evidence suggests that INF and ADA provide proven similar benefits in the treatment of childhood ACU, and they are both superior to ETA.

Association of knee injuries with accelerated knee osteoarthritis progression: data from the Osteoarthritis Initiative.

Abstract: While knee osteoarthritis is typically a slowly progressive disorder, it has recently been appreciated that 5 to 17% of knees have a rapid progression of structural damage (e.g. from normal to end-stage disease within 4 years) (1, 2). Characterization of the structural aspects of this phenomenon and its risk factors may provide insights into the nature of osteoarthritis progression and allow us to identify an at-risk subset for intervention. Identification of knee osteoarthritis phenotypes, such as those with accelerated knee osteoarthritis progression, may allow us to refine sampling for clinical studies (2-5) and develop interventions targeted at specific subtypes. Individuals with a history of joint trauma are 3 to 6 times more likely to develop knee osteoarthritis (6, 7) and are diagnosed approximately 10 years earlier than individuals without a history of joint trauma (8). Within 5 years of injury, knees have structural changes reflective of altered joint health (e.g., altered cartilage composition, altered bone structure) (9-12). Knee injuries are a strong risk factor for knee osteoarthritis and may distinguish knees with accelerated knee osteoarthritis from common knee osteoarthritis progression or knees with no knee osteoarthritis. We aimed to evaluate if a recent knee injury was associated with accelerated knee osteoarthritis. Furthermore, we conducted preliminary analyses to determine if participants with accelerated knee osteoarthritis progression, common knee osteoarthritis progression, and no knee osteoarthritis differed based on key baseline characteristics, which we selected a priori. These preliminary analyses helped us verify which variables should be adjusted for in our primary analyses.

Identifying Axial Spondyloarthritis in Dutch Primary Care Patients, Ages 20–45 Years, With Chronic Low Back Pain

Abstract: Objective To identify axial spondyloarthritis (SpA) in Dutch primary care patients with chronic low back pain (CLBP), and to design a simple referral model for general practitioners (GPs) that would identify patients at risk for axial SpA. Methods Patients (ages 20–45 years) with CLBP were identified from GP records. Assessments included inflammatory back pain questionnaires, medical interviews, physical examinations, HLA–B27, C-reactive protein level, conventional radiography, and magnetic resonance imaging. The outcome measure was axial SpA defined by the Assessment of SpondyloArthritis international Society (ASAS) criteria. Multivariable regression analysis with bootstrapping was used to develop the referral model. Results A total of 364 patients (mean ± SD age 36.3 ± 6.8 years) was recruited with a median symptom duration of 9.0 years. Eighty-six patients (24%) fulfilled the ASAS criteria for axial SpA. Of all potential determinants, the ASAS inflammatory back pain questionnaire, good response to nonsteroidal antiinflammatory drugs, family history of SpA, and symptom duration were identified as most relevant for diagnosing axial SpA by multivariable regression analysis related to axial SpA. The shrunken regression coefficients were, respectively, 1.04, 0.83, 0.73, and 0.23. The combination of these 4 items proved a useful area under the receiver operating characteristic curve of 0.75 (SE 0.03). In a simplified score model, at the suggested cutoff value of 1.5, the sensitivity was 83% and specificity was 59%. Conclusion This study shows that 1 of 4 primary care patients with CLBP was classified as having axial SpA. A preselection in primary care based on a combination of clinical items may be useful to facilitate the identification of patients at risk of axial SpA.

Is patellofemoral osteoarthritis common in middle-aged people with chronic patellofemoral pain?

Abstract: Objective To document the prevalence of radiographic osteoarthritis (OA) in the medial and lateral patellofemoral (PF) joint compartments relative to the prevalence of tibiofemoral (TF) joint OA in middle-aged and older adults with chronic PF knee pain. Methods A convenience sample of 224 people who volunteered for a clinical trial underwent weight-bearing posteroanterior and skyline knee radiographs of their most symptomatic eligible knee. Radiographic severity in the TF joint and in the medial and lateral PF joint compartments was independently graded by 2 examiners using the Kellgren/Lawrence (K/L) grading system. K/L grades ≥2 were considered evidence of OA. Results OA was common in this cohort, and the most prevalent pattern was combined TF joint and PF joint OA (n = 98 [44%]), followed by isolated PF joint OA (n = 57 [25%]). Isolated TF joint OA was rare. Overall, more people demonstrated radiographic OA in the PF joint (n = 155 [69%]) than in the TF joint (n = 100 [45%]). The majority of people with PF joint OA had OA in both the medial and lateral PF joint compartments (n = 98 [63%]). Even in people ages <50 years, radiographic OA was common (isolated PF joint OA, 26% [n = 21]; combined TF joint and PF joint OA, 29% [n = 23]). The severity of PF joint OA was similar across men and women. Conclusion PF joint OA was highly prevalent, more so than TF joint OA, and even in individuals ages <50 years. Further research is needed to elucidate the cause and effect relationship between chronic PF pain and PF joint OA.

Deficits in muscle mass, muscle density, and modified associations with fat in rheumatoid arthritis

Abstract: Objective To quantify muscle outcomes, independent of fat mass, in rheumatoid arthritis (RA) patients compared to healthy controls. Methods Quantitative computed tomography scans measured calf muscle and fat cross-sectional area (CSA) and muscle density (an index of intramuscular adipose tissue), and isometric dynamometry was used to measure ankle muscle strength in 50 participants with RA ages 18–70 years and 500 healthy controls. Multivariable linear regression models assessed muscle deficits in RA after adjusting for group differences in adiposity and assessing for an altered muscle–fat association. Associations between RA disease characteristics and fat-adjusted muscle outcomes were also assessed. Results Compared to controls, RA subjects had significantly greater body mass index (BMI) and fat area, and lower muscle area, muscle density, and muscle strength (P < 0.001 for all). Strength deficits were eliminated with adjustment for the smaller muscle area. The magnitude of muscle deficits, relative to controls, was significantly greater (P < 0.03 for interaction) in participants with lower fat area and BMI. Among those in the lower tertiles of adiposity, RA subjects demonstrated more significant deficits compared to controls with similar adiposity. In contrast, among those in the highest tertile for adiposity, RA was not associated with muscle deficits. Among RA, greater Sharp/van der Heijde scores were associated with lower muscle CSA and muscle density. Greater disease activity and disability were associated with low muscle density. Conclusion Deficits in muscle area and muscle density are present in RA patients compared to controls and are most pronounced in subjects with low fat mass. Greater joint destruction is associated with greater muscle deficits.

Autoantibodies to RuvBL1 and RuvBL2: A Novel Systemic Sclerosis–Related Antibody Associated With Diffuse Cutaneous and Skeletal Muscle Involvement

Abstract: Objective To identify and characterize a novel systemic sclerosis (SSc)–related autoantibody directed against a complex consisting of RuvBL1 and RuvBL2 (RuvBL1/2) and to assess its clinical correlations. Methods We first analyzed 316 consecutive patients with SSc who were evaluated at Kanazawa University Hospital. Controls included 290 patients with other connective tissue diseases, interstitial lung disease alone, or autoimmune hepatitis, and 50 healthy subjects. Autoantibody specificities were analyzed using RNA and protein immunoprecipitation assays. Autoimmune targets were affinity purified using patients' sera and subjected to liquid chromatography mass spectrometry. SSc patients in another institution in Japan and the University of Pittsburgh cohort were also included in analysis for evaluating clinical correlations. Results By protein immunoprecipitation assay, 6 SSc sera (1.9%) reacted with doublets with molecular weights of ∼50 kd. Liquid chromatography mass spectrometry of the partially purified autoantigen and additional immunoblot-based analyses revealed that this antibody specificity recognized RuvBL1/2. Anti-RuvBL1/2 antibody was exclusively detected in SSc patients. SSc patients with anti-RuvBL1/2 in both the Japanese and Pittsburgh cohorts consistently had higher frequencies of SSc in overlap with myositis and diffuse skin thickening than those without anti-RuvBL1/2. Compared with other autoantibodies related to SSc/myositis overlap (anti–PM-Scl and anti-Ku), anti-RuvBL1/2 was distinctive in terms of its associations with older age at SSc onset, male sex, and a high frequency of diffuse cutaneous involvement. Conclusion Anti-RuvBL1/2 antibody is a novel SSc-related autoantibody associated with a unique combination of clinical features, including myositis overlap and diffuse cutaneous involvement.

Clinical Characteristics of Children With Juvenile Dermatomyositis: The Childhood Arthritis and Rheumatology Research Alliance Registry

Abstract: Objective To investigate aspects of juvenile dermatomyositis (DM), including disease characteristics and treatment, through a national multicenter registry. Methods Subjects meeting the modified Bohan and Peter criteria for definite juvenile DM were analyzed from the cross-sectional Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry between 2010 and 2012 from 55 US pediatric rheumatology centers. Demographics, disease characteristics, diagnostic assessments, and medication exposure data were collected at enrollment. Results A total of 384 subjects met the criteria for analysis. At enrollment, the median Childhood Myositis Assessment Scale score was 51 (interquartile range [IQR] 46–52), the median Childhood Health Assessment Questionnaire score was 0 (IQR 0–0.5), and the median physician and subject global assessment scores were 1 (IQR 0–2) and 1 (IQR 0–3), respectively, out of a maximum of 10. Of the diagnostic assessments, magnetic resonance imaging was more likely than electromyography or muscle biopsy to show abnormalities. A total of 329 subjects had ≥2 diagnostic studies performed, and >34% of these subjects reported ≥1 negative study. Ninety-five percent had been treated with corticosteroids and 92% with methotrexate, suggesting that these medications were almost universally prescribed for juvenile DM in the US. Conclusion In 2 years, the ongoing CARRA Registry has collected clinical data on 384 children with juvenile DM and has the potential to become one of the largest juvenile DM cohorts in the world. More research is needed about prognostic factors in juvenile DM, and differences in therapy based on manifestations of disease need to be explored by practitioners. This registry provides the infrastructure needed to advance clinical and translational research and represents a major step toward improving outcomes of children with juvenile DM.