ERS/EULAR clinical practice guidelines for connective tissue diseases associated interstitial lung disease.

Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care.

Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies.

ERS/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung diseaseDeveloped by the task force for connective tissue disease-associated interstitial lung disease of the European Respiratory Society (ERS) and the European Alliance of Associations for Rheumatology (EULAR)Endorsed by the European Reference Network on rare respiratory diseases (ERN-LUNG).

Background: Interstitial lung disease (ILD) is a frequent manifestation of connective tissue diseases (CTDs) and is associated with high morbidity and mortality. Clinical practice guidelines to standardise screening, diagnosis, treatment and follow-up for CTD-ILD are of high importance for optimised patient care.

Methods: A European Respiratory Society and European Alliance of Associations for Rheumatology task force committee, composed of pulmonologists, rheumatologists, pathologists, radiologists, methodologists and patient representatives, developed recommendations based on PICO (Patients, Intervention, Comparison, Outcomes) questions with grading of the evidence according to the GRADE (Grading of Recommendations, Assessment, Development and Evaluations) methodology and complementary narrative questions agreed on by both societies.

Item selection for the development and validation of a revised classification criteria for adult and juvenile idiopathic inflammatory myopathies: MyoROC project.

Objective: A revision of the 2017 EULAR-ACR myositis classification criteria, namely EULAR-ACR funded Myositis Revision of Classification (MyoROC) project, is currently underway involving a large international group of experts. In the first phase of this project, we identified additional items to be tested in the criteria.

Methods: We distributed an electronic survey to International Myositis Assessment and Clinical Studies (IMACS) members to identify new items.

Shared genetic susceptibility between idiopathic inflammatory myopathies and common B cell lymphoma subtypes found primarily in the human leucocyte antigen region.

Objectives: To estimate shared genetic susceptibility between major subtypes of idiopathic inflammatory myopathies (IIM) and B cell lymphomas.

Methods: We paired summary statistics from genome-wide association studies (GWASs) of diffuse large B cell lymphoma, follicular lymphoma (FL), chronic lymphocytic leukaemia (CLL) and marginal zone lymphoma with those of dermatomyositis (DM) and polymyositis (PM) from a GWAS and an ImmunoChip study. We estimated local genetic correlation (r) for each disease pair using local analysis of (co)variant association (Bonferroni-corrected p value<0.

Immune Characterization of a Vietnamese Cohort With Idiopathic Inflammatory Myopathies.

Introduction/aims: Idiopathic inflammatory myopathies (IIMs) are classified into five subtypes that are associated with distinct groups of myositis-specific antibodies (MSAs). Variations in the prevalence, genetic predisposition and clinical manifestations exist in patients with IIM from different ethnic backgrounds. In this study, we aimed to characterize the immunopathological changes in muscle tissue and serum cytokines in a Vietnamese IIM cohort with Kinh ethnicity.

CD73 B-cell phenotypes and distinct cytokine profiles in patients with active anti-Jo-1 antibody positive idiopathic inflammatory myopathies.

Objectives: We performed multiparameter phenotyping of peripheral B cells in anti-Jo-1 antibody positive idiopathic inflammatory myopathies (IIM) to delineate disease-associated immunological profiles and the influence of B cells on disease activity.

Methods: Purified B cells from peripheral blood mononuclear cells from 16 patients with anti-Jo-1 antibody positive IIM (7 with untreated active IIM, 4 with active and treated IIM and 5 with inactive IIM) were analysed by multiparameter spectral flow cytometry. Dimensionality reduction and clustering analysis were applied to pre-gated CD19+B cells.

Modulating IL-21-driven B cell responses in idiopathic inflammatory myopathies via inhibition of the JAK/STAT pathway.

Background: Idiopathic inflammatory myopathies (IIM) are autoimmune disorders characterized by muscle inflammation and autoreactive B cell responses. The Janus kinase (JAK)-signal transducer and activator of transcription (STAT) signaling pathway is essential for B cell functions, making it a promising therapeutic target. This study explores the potential of tofacitinib, a JAK1/JAK3 inhibitor, to modulate B cell activity in IIM.

The role of multicriteria decision analysis in the development of candidate classification criteria for antisynthetase syndrome: analysis from the CLASS project.

Objectives: To develop and evaluate the performance of multicriteria decision analysis (MCDA)-driven candidate classification criteria for antisynthetase syndrome (ASSD).

Methods: A list of variables associated with ASSD was developed using a systematic literature review and then refined into an ASSD key domains and variables list by myositis and interstitial lung disease (ILD) experts. This list was used to create preferences surveys in which experts were presented with pairwise comparisons of clinical vignettes and asked to select the case that was more likely to represent ASSD.

Genetic Architecture of Idiopathic Inflammatory Myopathies From Meta-Analyses.

Objective: Idiopathic inflammatory myopathies (IIMs, myositis) are rare systemic autoimmune disorders that lead to muscle inflammation, weakness, and extramuscular manifestations, with a strong genetic component influencing disease development and progression. Previous genome-wide association studies identified loci associated with IIMs. In this study, we imputed data from two prior genome-wide myositis studies and analyzed the largest myositis data set to date to identify novel risk loci and susceptibility genes associated with IIMs and its clinical subtypes.

An update on autoantibodies in the idiopathic inflammatory myopathies.

Myositis-specific autoantibodies (MSAs) have become pivotal biomarkers for idiopathic inflammatory myopathies and have revolutionized understanding of the heterogeneous disease spectrum that affects both adults and children. The discovery and characterization of MSAs have substantially enhanced patient stratification based on clinical phenotype, thereby facilitating more precise diagnosis and ultimately improving management strategies. Advances in immunoassay technologies in the past 20 years have further propelled the field forward, enabling the detection of a growing repertoire of autoantibodies with high specificity and sensitivity; however, evolving research over the past decade has revealed that even within antibody-defined subsets, considerable clinical diversity exists, suggesting a broader spectrum of disease manifestations than previously acknowledged.

Efficacy and Safety of Subcutaneous Abatacept Plus Standard Treatment for Active Idiopathic Inflammatory Myopathy: Phase 3 Randomized Controlled Trial.

Objective: Our objective was to evaluate the efficacy and safety of subcutaneous (SC) abatacept and standard of care (SOC) for the treatment of idiopathic inflammatory myopathy (IIM) over 52 weeks.

Methods: In this randomized, double-blind, placebo-controlled phase III trial, patients with treatment-refractory IIM received SC abatacept (at 125 mg weekly) with SOC (abatacept group) or a placebo with SOC (placebo group). A 24-week double-blind period was followed by an open-label period to assess outcomes from continued therapy with abatacept and initiation with abatacept (placebo-to-abatacept switch group) from 24 to 52 weeks.

Autoantibodies against a subunit of mitochondrial respiratory chain complex I in inclusion body myositis.

Background: Autoantibodies are found in up to 80 % of patients with idiopathic inflammatory myopathies (IIM) and are associated with distinct clinical phenotypes. Autoantibodies targeting cytosolic 5'-nucleotidase 1A (anti-NT5C1A) are currently the only known serum biomarker for the subgroup inclusion body myositis (IBM), although detected even in other autoimmune diseases. The aim of the study was to identify new autoimmune targets in IIM.

Current efforts and historical perspectives on classification of idiopathic inflammatory myopathies.

Purpose Of Review: The classification of idiopathic inflammatory myopathies is challenging due to the large number of clinical, serological, histopathological and genetic findings, as well as the latest findings and developments in the field of myositis research. The latest official classification criteria are the 2017 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) classification criteria for adult and juvenile idiopathic inflammatory myopathies, which have been extensively reviewed in recent years for their applicability, sensitivity and specificity.

Recent Findings: The sensitivity and specificity of the 2017 ACR/EULAR criteria are sometimes performing better, but usually at the same level as the previous criteria.

Impact of cancer on the mortality of patients with idiopathic inflammatory myopathies by flexible parametric multistate modelling.

Background: Patients with idiopathic inflammatory myopathies (IIM) have an increased risk of cancer, but their cancer-related disease burden remains unclear.

Objectives: To explore how cancer might impact the mortality of patients with IIM and examine the associated prognostic factors for cancer and death.

Methods: We identified patients with IIM diagnosed between 1998 and 2020 and ascertained their cancer and death records via linkage to the Swedish healthcare and population registers.

The Dermatomyositis Disease Symptom Questionnaire (DM-DSQ): A Measure to Assess the Patient Experience of Dermatomyositis Symptoms.

Objective: Dermatomyositis (DM) symptoms negatively affect the quality of life of individuals living with the disease. Disease-specific, patient-reported outcome (PRO) instruments are needed to assess symptoms important to individuals with DM. This study aimed to conceptualize patient DM experience and disease activity definition to refine the development of the Dermatomyositis Disease Symptom Questionnaire (DM-DSQ), a novel PRO instrument capturing patient-reported symptoms.

The Role of Exercise to Improve Physiological, Physical and Psychological Health Outcome in Idiopathic Inflammatory Myopathies (IIM).

Idiopathic inflammatory myopathies (IIM) impact all aspects of health, physiological, physical, and psychological. Hallmark symptoms of IIM are muscle weakness, reduced muscle endurance and aerobic capacity. Recently, pain and fatigue as well as anxiety and depression have emerged as common and debilitating symptoms in patients with IIM.

Anti-FHL1 autoantibodies in adult patients with myositis: a longitudinal follow-up analysis.

Objectives: To determine prevalence and clinical associations of anti-Four-and-a-half-LIM-domain 1 (FHL1) autoantibodies in patients with idiopathic inflammatory myopathies (IIM) and to evaluate autoantibody levels over time.

Methods: Sera at the time of diagnosis from patients with IIM (n = 449), autoimmune disease controls (DC, n = 130), neuromuscular diseases (NMDs, n = 16) and healthy controls (HC, n = 100) were analysed for anti-FHL1 autoantibodies by enzyme-linked immunosorbent assay (ELISA). Patients with IIM FHL1+ and FHL1- were included in a longitudinal analysis.