OBJECTIVE To assess the long-term safety and effectiveness of tacrolimus for treating lupus nephritis (LN) in the real-world clinical setting. METHODS This is an ongoing, open-label, non-comparative, observational, post-marketing surveillance study conducted across 275 sites in Japan. Registered LN patients are being followed for 10 years. Here we report data relating to 5 years of tacrolimus maintenance therapy at the interim data cutoff in August 2016. RESULTS Of 1395 registered patients, 1355 received tacrolimus maintenance therapy for LN and provided safety data. The most common serious adverse drug reactions (ADRs) included pneumonia (1.1%), herpes zoster (1.0%), cellulitis (1.0%) and diabetes mellitus (1.0%). ADRs occurred mainly within the first 28 weeks of tacrolimus treatment, and no marked increase was observed during the follow-up period. Subgroup analyses suggested that risk factors for commonly observed ADRs associated with tacrolimus included inpatient management, LN disease severity, increasing age, abnormal renal or hepatic function, and comorbid or previous disease. The cumulative rate of progression to renal failure (based on the attending physician's assessment) was 0.8% at Year 1, and 6.6% at Year 5. Cumulative relapse rates were 7.8% and 30.6%, respectively. Urine proteincreatinine ratio, serum anti-dsDNA antibody levels, complement C3 levels, and steroid-sparing effect were all significantly improved from 4 weeks after tacrolimus treatment initiation (p less then 0.001), and were sustained over 5 years. CONCLUSION Long-term tacrolimus maintenance treatment over 5 years in the real-world clinical setting was well tolerated and effective in a large population of patients with LN. [Clinical trial registration number (www.ClinicalTrials.gov) NCT01410747].OBJECTIVE The aim of the present retrospective observational study was to evaluate in SSc patients the change of renal resistive index (RRI) over the time (ΔRRI) and under treatment as well as to correlate these changes with disease complications. METHODS 230 patients [29 male, median age 57 (48-67) years] were enrolled. At baseline and follow-up [3.43 (2.81-4.45) years] we collected following data disease variables, nailfold videocapilloscopy (NVC) pattern, FVC (Forced vital capacity), carbon oxide diffusing capacity (DLCO), systolic pulmonary arterial pressure (sPAP), presence of interstitial lung disease, RRI, evaluation of glomerular filtration rate (eGFR), new onset of pulmonary arterial hypertension (PAH). RESULTS RRI value is high in SSc patients with digital ulcers and ACA antibodies, active and late NVC patterns, lcSSc. A significant correlation was observed between ΔRRI and ΔsPAP (r=0.17, p=0.02), with statistically higher ΔRRI (0.08 ± 0.02 versus 0.03 ± 0.05, p=0.04) in patients complicated by PAH onset. No other new onset complication was associated with ΔRRI. The ROC curve analysis confirmed the predictive role of ΔRRI in development of new PAH (AUC 0.84; 95% CI 0.75-0.93, p=0.02). https://www.selleckchem.com/products/kt-413.html In SSc patients never exposed to sildenafil, ΔRRI was higher (0.04 ± 0.05) compared to both patients exposed to sildenafil during the study period (0.01±0.05, p=0.03) or in those exposed at the time of baseline evaluation (0.00 ± 0.05, p=0.01). CONCLUSION RRI and its variation in time are a reliable marker of SSc related vasculopathy, both in renal and extra-renal compartments.OBJECTIVE To determine the feasibility of comparing the Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans (CTPs) in treating new-onset, moderate juvenile dermatomyositis (JDM) using the CARRA registry, and to establish appropriate analytic methods to control for confounding by indication and missing data. METHODS A pilot cohort of 39 JDM patients from the CARRA registry was studied. Patients were assigned by the treating physician, considering patient/family preferences, to one of three CTPs methotrexate and prednisone (MP), intravenous methylprednisolone, methotrexate and prednisone (MMP) or intravenous methylprednisolone, methotrexate, prednisone and intravenous immunoglobulin (MMPI) .The primary outcome was the proportion of patients achieving moderate improvement at 6 months under each CTP. Statistical methods including multiple imputation and inverse probability of treatment weighting were used to handle missing data and confounding by indication. RESULTS Patients received MP (n=13), MMP (n=18) and MMPI (n=8). Patients in all CTPs had significant improvement in disease activity. Of the 36 patients who remained in the pilot study at 6 months, 16 (44%) of them successfully achieved moderate improvement at 6 months (6/13, 46% for MP; 7/15, 47% for MMP; 3/8, 38% for MMPI).After correcting for confounding there were no statistically significant pairwise differences between the CTPs (p = 0.328-0.88). CONCLUSION We gained valuable experience and insight from the pilot study to guide the design and analysis of comparative effectiveness studies using the CARRA registry CTP approach. Our analytical methods can be adopted for future comparative effectiveness studies and applied to other rare disease observational studies.OBJECTIVE Burnout among physicians is common and has important implications. We assessed the extent of burnout among rheumatology practitioners and its associations. METHODS 128 attendees at the 2019 Rheumatology Winter Clinical Symposium were surveyed using the Maslach Burnout Index™ (MBI™) and a demographics questionnaire. Scores for emotional exhaustion (EE) ≥27, depersonalization (DP) ≥10, and personal accomplishment (PA) ≤33 were considered positive for burnout. Data regarding practitioner characteristics including age, sex, years in practice, and other demographics of interest were also collected. These data were used to determine prevalence and interactions of interest between practitioner characteristics and the risk of burnout. RESULTS Of the 128 respondents, 50.8% demonstrated burnout in at least one MBI™ domain. Dissatisfaction with EMR was associated with a 2.86 times increased likelihood of burnout (OR=2.86 p=0.015, 95% CI 1.23-6.65). Similar results were found for lack of exercise (OR=5.00 p= 0.