The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a clinical tool that quantifies disease activity and damage in cutaneous lupus erythematosus (CLE). The activity score is based on the degree of erythema, scale, mucous membrane lesions, and nonscarring alopecia.¹ Unlike other outcome measures in dermatology, CLASI scores are not based solely on the area of involved skin; rather, parts of the body that are most visible are weighted more heavily than those that are usually covered.¹ The CLASI has already been shown to have good content validity, addressing the most relevant aspects of CLE as determined by an expert panel of dermatorheumatologists.¹ It also has good interrater and intrarater reliability when used by either dermatologists or rheumatologists.^1,2 Early small clinical studies have demonstrated responsiveness in all subsets of CLE, including individual lesions, localized and generalized discoid lupus erythematosus (DLE), as well as subacute cutaneous lupus erythematosus (SCLE) and tumid lupus erythematosus.^3-7

Objective: Anti‐synthetase syndrome (ASSD) is a rare systemic autoimmune rheumatic disease (SARD) with significant heterogeneity and no shared classification criteria. We aimed to identify clinical and serological features associated with ASSD that may be suitable for inclusion in the data‐driven classification criteria for ASSD.Methods: We used a large, international, multicenter “Classification Criteria for Anti‐synthetase Syndrome” (CLASS) project database, which includes both patients with ASSD and controls with mimicking conditions, namely, SARDs and/or interstitial lung disease (ILD). The local diagnoses of ASSD and controls were confirmed by project team members. We employed univariable logistic regression and multivariable Ridge regression to evaluate clinical and serological features associated with an ASSD diagnosis in a randomly selected subset of the cohort.Results: Our analysis included 948 patients with ASSD and 1,077 controls. Joint, muscle, lung, skin, and cardiac involvement were more prevalent in patients with ASSD than in controls. Specific variables associated with ASSD included arthritis, diffuse myalgia, muscle weakness, muscle enzyme elevation, ILD, mechanic's hands, secondary pulmonary hypertension due to ILD, Raynaud phenomenon, and unexplained fever. In terms of serological variables, Jo‐1 and non–Jo‐1 anti‐synthetase autoantibodies, antinuclear antibodies with cytoplasmic pattern, and anti‐Ro52 autoantibodies were associated with ASSD. In contrast, isolated arthralgia, dysphagia, electromyography/magnetic resonance imaging/muscle biopsy findings suggestive of myopathy, inflammatory rashes, myocarditis, and pulmonary arterial hypertension did not differentiate between patients with ASSD and controls or were inversely associated with ASSD.Conclusion: We identified key clinical and serological variables associated with ASSD, which will help clinicians and offer insights into the development of data‐driven classification criteria for ASSD. image

Objectives: This randomized, placebo-controlled pilot trial evaluated the efficacy and safety of abatacept in patients with anti-synthetase syndrome-associated interstitial lung disease (ASyS-ILD). Methods: Participants with active ASyS-ILD were randomized to receive abatacept (n = 9) or placebo (n = 11) for 24 weeks, followed by a 24-week open-label extension with abatacept for all participants. The primary endpoint was a change in % predicted forced vital capacity (%FVC) from baseline to week 24. Secondary endpoints included changes in the FVC (ml), % predicted diffusing capacity of the lung for carbon monoxide (%DLCO), shortness of breath questionnaire (SOBQ) and pulmonary disease activity on a visual analogue scale (VAS) at weeks 24 and 48. Pre-post baseline analysis of FVC and quantitative image analysis (QIA) of high-resolution computed tomographic scans were performed. Data were analysed using a generalized linear mixed model. The study was not powered for primary or secondary endpoints. Results: At week 24, there was no significant difference in the primary endpoint of %FVC change between abatacept and placebo (between treatment difference of −0.35, 95%CI −6.91 to 6.21, P = 0.914) and in all secondary endpoints. However, by week 48, trends favouring abatacept in %FVC, FVC (ml), %DLCO and SOBQ were observed without statistical significance. There was a significant improvement in pulmonary disease activity VAS and pre-post baseline slopes of %FVC and QIA scores in the abatacept arm. Abatacept was generally well tolerated. Conclusion: Abatacept did not significantly improve %FVC at 24 weeks. However, trends at 48 weeks suggest potential benefits, supporting the need for a larger, long-term randomized controlled trial. Clinical trial registration: clinicaltrials.gov; NCT03215927

Magnetic resonance imaging (MRI) has emerged as a key non-invasive tool for the evaluation of idiopathic inflammatory myopathies (IIM); however, heterogeneity in techniques, protocols, and grading systemics impedes standardization. This scoping review systematically examined the MRI techniques, protocols, and grading systems reported in the adult IIM literature. A systematic search of PubMed, EMBASE, and Cochrane databases was conducted from 2000 to 2024 using keywords related to IIM and MRI. Studies involving adults with IIM who underwent MRI were screened and reviewed for inclusion. Forty-nine studies were included in the analysis, 13 of which evaluated whole-body MRI and 36 evaluated dedicated body-part MRI, collectively reporting data from 2810 IIM patients. A wide range of imaging protocols was observed with variations in scanner type, field strength, sequence combinations, and anatomical coverage. Semi-quantitative visual grading was the most commonly used assessment method (31/49, 63.2 %), with binary scoring in 23/31 and software-assisted or automated techniques in 8/31. Six studies used descriptive analysis alone. Inter-rater agreement was reported in 15 studies, with variable reliability observed for both muscle edema (intraclass correlation coefficient [ICC] range: 0.78-1.00; kappa range: 0.30-1.00) and replacement of skeletal muscle by fat (ICC range: 0.77-0.97; kappa range: 0.54-0.93). Several studies have reported that WB-MRI patterns correlate with clinical measures of disease activity and can discriminate between myopathic diseases and IIM subtypes. In summary, despite the clinical utility of MRI for IIM, significant methodological variability remains. Future research should focus on standardizing protocols and grading systems to enhance the consistency and reliability of MRI assessments for IIM.