Objective:This study aims to compare the frequency of instances in which the physician's global assessment of disease activity (PhGA) was scored >0 despite the absence of active joints in children with juvenile idiopathic arthritis (JIA), using two multicenter patient datasets and one single-center dataset from a pediatric rheumatology center with expertise in clinimetric assessments. Methods:Data were extracted from two multicenter datasets and one single-center dataset, comprising 9,081, 563, and 394 patients, respectively. Patients with an active joint count (AJC) of 0 were included. The PhGA and fulfillment of other criteria from the 2004 or 2011 Wallace definition of clinically inactive disease (CID) were assessed. UpSet plots were used to analyze the frequency and overlap of PhGA and CID items across the datasets. Results:Among patients with an AJC of 0, the percentage for whom a PhGA score >0 was the sole unmet CID criterion was 14.8% and 13.7% in the two multicenter datasets and 5.1% in the single-center dataset. The CID criteria that were most frequently not met when the PhGA was scored >0 were elevated acute-phase reactants (APRs) and morning stiffness lasting ≥15 min. Conclusion:The discordance between the absence of active joints and a PhGA score >0 was less common in the single-center sample, suggesting that regular use and training may increase concordance between PhGA and AJC in patients without clinical signs of joint disease. APR elevation and parent-/patient-reported morning stiffness seemed to play a major role in prompting physicians to assign a non-zero global score.

Teriflunomide is widely used as an active comparator in Phase 3 randomised trials for relapsing multiple sclerosis (RMS). Temporal changes in disease activity within teriflunomide-treated cohorts have not been systematically examined. To assess temporal trends in relapse and disability outcomes across teriflunomide arms of Phase 3 multiple sclerosis (MS) trials and identify predictors of between-trial heterogeneity. We performed a systematic review and meta-analysis of Phase 3 randomised controlled trials including a teriflunomide arm. PubMed, Scopus, and ClinicalTrials.gov were searched up to October 2025. Annualised relapse rate (ARR) and 12- and 24-week confirmed disability worsening (CDW) were extracted together with baseline characteristics. Risk of bias was assessed using the Cochrane Risk of Bias 2 tool. Random-effects meta-analyses, meta-regression, and sensitivity analyses were performed. Twelve teriflunomide cohorts from eight trials involving 4,900 adults with RMS were included. ARR ranged from 0.11 to 0.37 with substantial heterogeneity (I2 = 94

OBJECTIVE:Juvenile dermatomyositis (JDM) is a rare autoimmune condition. The treat-to-target strategy has garnered interest in pediatric rheumatology. It is based on defining clear therapeutic targets, with frequent disease activity monitoring, and adjustment of the treatments if targets are not met within a defined time frame. Recently, an international task force of experts launched an initiative aimed at the development of recommendations for the adoption of treat-to-target strategy in JDM. This study was done to support those recommendations. We aimed to determine the time-to-treatment response in patients with JDM, to better inform the development of a treat-to-target strategy in JDM. METHODS:This is a retrospective review of patients with a physician-confirmed diagnosis of JDM, observed at two tertiary care centers-the Istituto di Ricovero e Cura a Carattere Scientifico (IRCCS) Istituto Giannina Gaslini (Gaslini), and The Hospital for Sick Children (SickKids). Demographic and clinical data were obtained on all patients with JDM during the first two years following diagnosis. Kaplan-Meier survival curves were used to determine time to outcome definitions. RESULTS:A total of 187 patients were identified across two sites; the mean age of diagnosis was 8 years. On average, patients with JDM achieved normalization of muscle enzymes and muscle remission three months and six months after treatment initiation, respectively. Skin remission occurred within 12 months after starting treatment. Time to reach inactive disease varied between the sites, with median time being 10.3 months (Gaslini) and 8.8 months (SickKids). CONCLUSION:This study provides real-world data for potential timelines to target with a treat-to-target strategy for JDM.

Disability worsening is the critical long-term outcome in multiple sclerosis, yet the Expanded Disability Status Scale incompletely captures neurological deterioration and has limited sensitivity in the short time windows of clinical trials. Composite endpoints incorporating functional measures have been proposed to address these limitations, but whether they reliably improve detection of treatment effects has not been established across trials. We conducted a post-hoc analysis of individual patient data from ten phase III randomised controlled trials (ASCEND, BRAVO, CONFIRM, DEFINE, EXPAND, INFORMS, OLYMPUS, OPERA I/II, and ORATORIO; n = 9,369), spanning relapsing-remitting and progressive multiple sclerosis. Confirmed disability worsening was defined using harmonised criteria with the msprog package and confirmed at 24 weeks. Treatment effects were estimated using Cox proportional hazards models and combined across trials in a one-stage individual patient data framework. Composite endpoints were constructed from the Expanded Disability Status Scale, the timed 25-foot walk test, and the nine-hole peg test using logical unions (OR-type), intersections (AND-type), and majority-vote structures. Sensitivity to treatment effect was quantified using Z-scores (the ratio of the pooled log-hazard ratio to its standard error) and compared to the Expanded Disability Status Scale reference using interaction tests. Event rates varied across components: the timed walk test generated the highest rates (up to 46.8%) while the nine-hole peg test generated the lowest (as low as 2.1%). OR-type composite endpoints showed weaker treatment effects than the Expanded Disability Status Scale alone, with the largest reductions in sensitivity observed for endpoints incorporating the timed walk test (ΔZ up to +2.26; interaction p = 0.004). These findings were confirmed across disease subtypes and were pronounced in relapsing-remitting trials, where no composite endpoint outperformed the Expanded Disability Status Scale. In progressive multiple sclerosis, the combination of the Expanded Disability Status Scale and the nine-hole peg test showed numerically stronger treatment effects (ΔZ = −1.65), though interaction tests did not reach statistical significance (p = 0.051). Composite endpoints do not systematically improve treatment effect detection in multiple sclerosis trials. Increased event capture driven by the timed walk test introduces noise that dilutes the treatment signal rather than amplifying it, highlighting that event rate and endpoint quality are not interchangeable. Upper limb function assessed by the nine-hole peg test provides complementary and specific information, particularly in progressive disease. The combination of global disability and upper limb measures represents a promising direction for future endpoint development in progressive multiple sclerosis trials, warranting validation. ### Competing Interest Statement Francesca Bovis and Noemi Montobbio report no competing interest. Alessio Signori received speakers honoraria from Chiesi, Novartis, and Horizon and grant from MSBase, Tomas Kalincik served on scientific advisory boards for MS International Federation and World Health Organisation, BMS, Roche, Janssen, Sanofi Genzyme, Novartis, Merck and Biogen, steering committee for Brain Atrophy Initiative by Sanofi Genzyme, received conference travel, support and/or speaker honoraria from WebMD Global, Eisai, Novartis, Biogen, Roche, Sanofi-Genzyme, Teva, BioCSL and Merck and received research or educational event support from Biogen, Novartis, Genzyme, Roche, Celgene and Merck. Douglas L Arnold reports consulting honoraria from Alexion, Biogen, Celgene, Frequency Therapeutics, GENeuro, Genentech, Merck/EMD Serono, Novartis, Roche, and Sanofi; and ownership interest in NeuroRx. Mar Tintore has received compensation for consulting services, speaking honoraria and research support from Almirall, Bayer Schering Pharma, Biogen-Idec, Genzyme, Immunic Therapeutics, Janssen, Merck-Serono, Novartis, Roche, Sanofi-Aventis, Viela Bio and Teva Pharmaceuticals. Data Safety Monitoring Board for Parexel and UCB Biopharma, Relapse Adjudication Committee for IMCYSE SA. Ludwig Kappos reports receiving honoraria for consulting and/or advisory board services for Actelion, Bayer, BMS, df-mp Molnia & Pohlmann, Celgene, Eli Lilly, EMD Serono, Genentech, Glaxo Smith Kline, Janssen, Japan Tobacco, Merck, MH Consulting, Minoryx, Novartis, F. Hoffmann-La Roche Ltd, Senda Biosciences Inc., Sanofi, Santhera, Shionogi BV, TG Therapeutics, and Wellmera; license fees for Neurostatus UHB products; and grants from Novartis, Innosuisse, and Roche. Maria Pia Sormani reports receiving honoraria for consulting and/or advisory board/lecture services from Biogen, Celgene, GeNeuro, GSK, Immunic, Medday, Merck, Novartis, Roche, and Sanofi. ### Funding Statement This study did not receive any funding ### Author Declarations I confirm all relevant ethical guidelines have been followed, and any necessary IRB and/or ethics committee approvals have been obtained. Yes The details of the IRB/oversight body that provided approval or exemption for the research described are given below: All the data have been de-identified. I confirm that all necessary patient/participant consent has been obtained and the appropriate institutional forms have been archived, and that any patient/participant/sample identifiers included were not known to anyone (e.g., hospital staff, patients or participants themselves) outside the research group so cannot be used to identify individuals. Yes I understand that all clinical trials and any other prospective interventional studies must be registered with an ICMJE-approved registry, such as ClinicalTrials.gov. I confirm that any such study reported in the manuscript has been registered and the trial registration ID is provided (note: if posting a prospective study registered retrospectively, please provide a statement in the trial ID field explaining why the study was not registered in advance). Yes I have followed all appropriate research reporting guidelines, such as any relevant EQUATOR Network research reporting checklist(s) and other pertinent material, if applicable. Yes Individual patient data from these RCTs were made available through the International Progressive MS Alliance (IPMSA) project (award reference number PA-1603-08175). Access requests should be forwarded to the relevant data controllers.

OBJECTIVES:The aim of this study was to evaluate whether the treatment effects of tolebrutinib on confirmed disability worsening (CDW) diverge from its effects on relapse prevention compared with other disease-modifying therapies (DMTs) for relapsing multiple sclerosis (MS). METHODS:We extracted published effect estimates for annualized relapse rate (ARR) and CDW confirmed at 24 weeks from all phase 3 trials with teriflunomide as the active comparator: ASCLEPIOS (ofatumumab), OPTIMUM (ponesimod), ULTIMATE (ublituximab), EVOLUTION (evobrutinib), and GEMINI (tolebrutinib). When duplicate trials were available, pooled estimates were derived. Log-transformed estimates were used in a weighted linear regression of CDW vs ARR, with bubble size reflecting statistical precision. Tolebrutinib was excluded from the regression fit but displayed for comparison. RESULTS:Across 4 DMTs other than tolebrutinib, a strong linear association was observed between treatment effects on ARR and CDW (R2 = 0.997), indicating that disability benefit was generally proportional to relapse reduction. By contrast, tolebrutinib deviated from this relationship, with a hazard ratio for CDW of 0.71 (95% CI 0.53-0.95) despite a relapse rate ratio of 1.03 (95% CI 0.85-1.25). DISCUSSION:Tolebrutinib was the only therapy to show a benefit on CDW without a measurable effect on relapses, highlighting a dissociation between disability worsening and relapse suppression not observed with other DMTs.

BACKGROUND AND OBJECTIVES:Neuromyelitis optica spectrum disorder (NMOSD) is a severe condition usually associated with aquaporin-4 (AQP4) antibodies. A clinical presentation suggestive of NMOSD can also be associated with myelin oligodendrocyte glycoprotein (MOG) antibodies (MOGAD). NMOSD can be diagnosed in the absence of autoantibodies (double-negative NMOSD [DN-NMOSD]), but this subgroup has been poorly investigated. We conducted a systematic review and meta-analysis to define the clinical spectrum, prognosis, and treatment response in DN-NMOSD vs AQP4-NMOSD/MOGAD. METHODS:We searched on PubMed, Scopus, Embase, Google Scholar, Cochrane Library, and ClinicalTrials.gov databases of studies on patients fulfilling inclusion criteria. Patient characteristics, outcome measures, and treatment regimens were extracted. RESULTS:We included 41 of 1,027 articles screened and analyzed 671 patients with DN-NMOSD (median age 38.6 years [range IQR: 32.5-42.85]; female-to-male ratio 1.5:1; median follow-up 44.4 months [range 1-600]), 73.6% of which relapsed. In the meta-analysis, mean annualized relapse rate (ARR) was higher, albeit not significantly, in DN-NMOSD (1.08; 95% CI 0.73-1.43) vs AQP4-NMOSD (0.84; 95% CI 0.45-1.23) and MOGAD (0.61; 95% CI 0.39-0.83, p = 0.08). Administration of maintenance immunosuppression in DN-NMOSD led to a significant ARR reduction (pooled rate ratio 0.19, 95% CI 0.07-0.49; p = 0.001), with high heterogeneity (I2 = 90%, p < 0.0001). In meta-regression, no covariates were associated with ARR reduction, including the administration of specific drugs (rituximab, p = 0.288; azathioprine, p = 0.291; mycophenolate, p = 0.918). The pooled mean difference in pre‑ and post‑maintenance treatment Expanded Disability Status Scale values indicated a significant change in disability in MOGAD (-0.93, 95% CI -1.67 to -0.19, p = 0.02) but not in AQP4-NMOSD (-0.62, 95% CI -1.85 to 0.61, p = 0.27) or DN-NMOSD (-0.52 (95% CI -1.30 to 0.25, p = 0.16). DISCUSSION:DN-NMOSD is a heterogenous, severe and highly relapsing disease, where attacks lead to irreversible dysfunction. The administration of maintenance immunotherapy reduces the relapse risk and should be considered early to prevent further disability.

BACKGROUND:Spinal muscular atrophy (SMA) is a genetic neuromuscular disorder caused by survival motor neuron (SMN1) deletion. While loss of ambulation in SMA type III typically occurs at a median age of 13.4 years, outcomes in the treatment era remain unclear. This study aims to address that gap by investigating ambulation outcomes in individuals with type III receiving disease-modifying therapies. METHODS:This retrospective study analysed prospectively collected international data. Time-dependent Cox models assessed the association between treatment initiation and age at loss of ambulation, adjusting for age at onset, sex, SMN2 copies, birth year and country. Treatment was modelled as a time-dependent covariate to avoid immortal time bias. Descriptive analyses used Mann-Whitney U and χ² tests. RESULTS:Among 555 individuals with type III, treatment halved the risk of ambulation loss (HR=0.50), with median loss at 44 vs 32 years in treated and untreated groups. Later onset, ≥4 SMN2 copies and female sex were also protective. The treatment effect was significant in type IIIA (HR=0.34) but not IIIB, with no significant interactions by sex, country or SMN2, though effects remained directionally protective. CONCLUSIONS:Treatment in type III reduced the risk of ambulation loss by 50%, extending median ambulation by 12 years, with the greatest benefit in type IIIA. Later onset, female sex and higher SMN2 copy number were also protective but did not modify treatment effect. These findings underscore the value of early treatment and support its broad use to preserve ambulation across clinical subgroups.

Importance:Live attenuated measles-mumps-rubella (MMR) and varicella vaccines are recommended for immunocompetent people with multiple sclerosis (MS) lacking immunity. However, concerns about postvaccination relapses may contribute to vaccine hesitancy, and robust data addressing this risk remain limited. Objective:To assess whether the risk of MS relapse within 1 year after MMR or varicella vaccination is not unacceptably worse than risk after no such vaccination based on a predefined noninferiority margin. Design, Setting, and Participants:This cohort study used prospectively collected data from July 2016 to October 2024 for people with MS at a tertiary MS center in Barcelona, Spain, who received at least 1 dose of live attenuated MMR and/or varicella vaccine due to serologic susceptibility. Vaccinated patients were matched 1:2 with unvaccinated control individuals with MS by sex, age, and epoch of first demyelinating event. Exposures:Live attenuated MMR and/or varicella vaccine. Main Outcomes and Measures:Primary outcomes were relapse count and time to first relapse within 1 year of time 0, analyzed using Poisson and Cox proportional hazards regression models with inverse probability weighting based on a propensity score including treatment category, Expanded Disability Status Scale score, and annualized relapse rate in the prior year. Noninferiority was predefined as an annualized relapse rate ratio with an upper bound of the 95% CI less than 1.4. Results:A total of 369 people with MS were included, of whom 123 were vaccinated (mean [SD] age, 28.75 [8.72] years; 85 females [69.1%]) and matched to 246 unvaccinated controls (mean [SD] age, 28.66 [8.60] years; 170 females [69.1%]). Overall, 36 relapse events were observed (15 [41.7%] among vaccinated patients and 21 [58.3%] among unvaccinated controls). In the weighted Poisson model, although relapse incidence was not significantly different between vaccinated and unvaccinated individuals (incidence rate ratio, 0.52; 95% CI, 0.23-1.06), the noninferiority criterion was met. Similarly, weighted Cox proportional hazards regression models showed no difference in hazard of relapse between groups (hazard ratio [HR], 0.55; 95% CI, 0.25-1.17) but the noninferiority criterion was met. Sensitivity and exploratory analyses, including time-dependent adjustment for postbaseline treatment exposure (HR, 0.60; 95% CI, 0.27-1.33), restriction to the high-risk period (n = 15 events), and comparisons of magnetic resonance imaging before (n = 43 events) and after (n = 21 events) exposure in vaccinated patients, revealed findings consistent with those of the primary analysis. Conclusions and Relevance:In this cohort study of nonimmunosuppressed people with MS, vaccination with live attenuated MMR or varicella vaccine was not associated with increased risk of postvaccination relapse. The results support the administration of these vaccines when indicated and may help reassure clinicians and patients, reducing vaccine hesitancy.

Objective To develop evidence-based criteria to classify patients with syndrome of undifferentiated recurrent fevers (SURF).Methods One hundred twelve patients with SURF observed in a single tertiary referral center were analyzed. Patients with genetically confirmed hereditary recurrent fever (HRF) or with periodic fever, aphthosis, pharyngitis, and adenitis (PFAPA) syndrome already analyzed for the Eurofever classification criteria were used as disease controls. A decision tree approach was tested by randomly splitting the available data in a training set and in an internal test set. An alternative model using a classical regression model was also analyzed. An external validation for both approaches was performed on 123 patients recruited from four other centers.Results The decision tree model integrating clinical and genetic data identified 91% of patients with SURF. A decision tree model based solely on clinical variables identified up to 88% of patients with SURF. The logistic regression model including genetic tests exhibited an overall accuracy of 89.2% (95% confidence interval [CI] 81.1-94.7). In contrast, the logistic regression model exclusively based on clinical manifestations displayed an overall accuracy of 66.7% (95% CI 56.1-76.1). When the classification criteria including genetic tests were applied to the external validation cohort, the model demonstrated a strong discriminative power, with areas under the receiver operating characteristic curve of 96.3% using the decision tree model and 88.0% with the logistic regression model.Conclusion The study shows the possibility of achieving evidence-based criteria that can classify SURF at least with respect to the main HRF and PFAPA syndrome and may be considered as a preliminary tool for the enrollment of more homogeneous cohorts of patients in future studies.

ObjectivesThe aim of this study was to evaluate whether the treatment effects of tolebrutinib on confirmed disability worsening (CDW) diverge from its effects on relapse prevention compared with other disease-modifying therapies (DMTs) for relapsing multiple sclerosis (MS).MethodsWe extracted published effect estimates for annualized relapse rate (ARR) and CDW confirmed at 24 weeks from all phase 3 trials with teriflunomide as the active comparator: ASCLEPIOS (ofatumumab), OPTIMUM (ponesimod), ULTIMATE (ublituximab), EVOLUTION (evobrutinib), and GEMINI (tolebrutinib). When duplicate trials were available, pooled estimates were derived. Log-transformed estimates were used in a weighted linear regression of CDW vs ARR, with bubble size reflecting statistical precision. Tolebrutinib was excluded from the regression fit but displayed for comparison.ResultsAcross 4 DMTs other than tolebrutinib, a strong linear association was observed between treatment effects on ARR and CDW (R2 = 0.997), indicating that disability benefit was generally proportional to relapse reduction. By contrast, tolebrutinib deviated from this relationship, with a hazard ratio for CDW of 0.71 (95% CI 0.53-0.95) despite a relapse rate ratio of 1.03 (95% CI 0.85-1.25).DiscussionTolebrutinib was the only therapy to show a benefit on CDW without a measurable effect on relapses, highlighting a dissociation between disability worsening and relapse suppression not observed with other DMTs.

BACKGROUND:Reproducibility and comparability of disability outcomes remain major challenges in multiple sclerosis (MS) research. Definitions of disability progression vary widely across studies, and calculation criteria are often insufficiently documented to enable replication. FRAMEWORK:To address these issues, a consensus process endorsed by the International Advisory Committee on Clinical Trials in MS was conducted to develop practical guidelines for outcome calculation across a range of study designs and research questions. Instead of proposing a single universal endpoint definition, we establish specific recommendations for each use case, within a fixed parameter space derived from extraction and systematisation of common practices for baseline selection, event confirmation, relapse handling, and event categorisation. These criteria are implemented in a shared computational framework, "msprog," available as open-source R and Python software and as a web application. The tool supports different disability scales, facilitates full reporting of parameters, and adapts to both clinical trial and real-world settings. CONCLUSION:The integration of consensus-based guidelines with a standardised, extensible implementation provides a practical basis for consistent and reproducible specification of disability outcomes in MS.

ABSTRACTAimIdentify values that could predict the presence of increased pressure‐pain sensitivity independent of the migraine cycle through a single assessment.MethodsThis was a secondary analysis of a previous study in which 198 episodic and chronic migraine patients were assessed during all phases of the migraine cycle. Pressure pain threshold (PPT) was assessed over the temporalis, cervical spine, hand, and leg. Migraine patients were divided into two sub‐groups: patients with increased pressure‐pain sensitivity (IPS) and with No IPS (No‐IPS). A Chi‐squared Automatic Interaction Detection decision tree analysis was used to identify predictors to be included in the IPS or NoIPS group. To assess the internal validity of the model, a tenfold cross‐validation was applied.Results161 (81%) patients were included in the IPS group, while 37 (19%) in the NoIPS group. Migraine patients with: (1) Temporalis PPT ≤ 130 kPa; (2) Temporalis PPT > 130 kPa and ≤ 197.5 kPa and hand PPT ≤ 347.33 kPa; (3) Temporalis PPT > 197.5 kPa and hand PPT ≤ 315 kPa; were correctly included in the IPS group with a sensitivity of 96%, a specificity of 81%, a positive predictive value of 96%, and a negative predictive value of 81%. The accuracy of the model and the cross‐validation analysis were respectively 93% and 92%.ConclusionThe high internal validity suggests that our model could precisely predict the presence of IPS independently by the phase in which the assessment occurred. Trigeminal and hand PPT cut‐off values could be used to identify patients with IPS.

BACKGROUND:Interest in progression independent of relapse activity (PIRA) as an endpoint in multiple sclerosis (MS) clinical trials is surging. However, established definitions of PIRA may produce biased treatment effect estimates in the presence of a treatment-induced relapse reduction. METHODS:We applied different definitions of PIRA to pooled data from the OPERA I/II clinical trials (clinicaltrials.gov identifiers: NCT01247324, NCT01412333). Treatment effects on PIRA according to different methods were quantified by hazard ratios (HRs) and risk ratios (RRs). Next, we evaluated the bias in each definition using synthetic Expanded Disability Status Scale (EDSS) data simulating a control and an experimental arm with varying treatment effects on relapses and on PIRA. We quantified the bias by comparing the estimated effect on PIRA with the known true effect. FINDINGS:The pooled OPERA I/II population included 1656 participants. Estimated treatment effects on PIRA varied from a non-significant HR of 0.83 (CI = 0.66-1.04) to an HR of 0.73 (CI = 0.59-0.90) depending on the definition used. Follow-up analyses on simulated data (n = 800 per arm) revealed an underestimation of the true treatment effect on PIRA when using established definitions, with increasing bias as treatment effect on relapses increased. Defining PIRA as complementary to relapse-associated worsening (RAW) provided a less biased and operationally simple alternative. INTERPRETATION:For clinical trials with PIRA as an endpoint, we suggest a "complementary" definition of PIRA, relying on accurate exclusion of RAW promoted by appropriate visit timing. FUNDING:Italian Ministry of University and Research.

OBJECTIVES:Despite the recent prognostic improvement, a sizeable proportion of patients with juvenile dermatomyositis (JDM) respond suboptimally to contemporary therapies. This study aimed to develop recommendations for treating JDM to target. METHODS:A Steering Committee formulated a set of provisional recommendations based on evidence derived from a systematic literature review and a retrospective chart review of patients. These were discussed, amended, and voted on by an international Task Force, including 28 paediatric rheumatologists, 2 specialists in neuromuscular diseases, 1 dermatologist, 1 physical therapist, 1 research nurse, 2 patients with JDM, and 1 parent of a patient with JDM. Items that achieved at least an 80% majority vote were accepted as final recommendations. RESULTS:Although the literature review did not reveal trials that compared a treat-to-target strategy with a nonsteered approach, it provided indirect evidence about specific end points that could serve as targets that facilitated development of recommendations. The group reached consensus on 7 overarching principles and 12 recommendations. It was agreed that both patients/parents and treaters should share decisions in setting treatment targets and therapeutic strategies, with inactive disease as the preferred target and minimal disease activity an alternative one. Inactive disease is targeted to be achieved within 12 months after treatment start. Interim targets include minimal and moderate clinical improvement within 6 weeks and 3 months, respectively, and normalisation of muscle strength within 6 months. High-dose glucocorticoids remain fundamental in the initial management, but progressive tapering and discontinuation within 12 months through optimisation of concomitant immunomodulatory therapy was advised. A research agenda was formulated. CONCLUSIONS:The Task Force developed recommendations for treating JDM to target, being aware that the evidence is not strong and needs to be expanded by future research. Implementation of the recommendations in clinical practice will help to reach optimal outcomes for JDM.