Risk prediction model for relapse of anti-synthetase syndrome- associated interstitial lung disease in Japanese multicentre MYKO cohort study.

Objectives: This study aimed to establish a risk prediction model for the relapse of anti-synthetase syndrome-associated interstitial lung disease (ASyS-ILD).

Methods: Patients diagnosed with ASyS-ILD and treated with prednisolone and calcineurin inhibitors as remission induction therapy were enrolled in the Japanese multicentre MYKO cohort. We followed up on patients who experienced relapse of ASyS-ILD after remission induction therapy, and examined the risk factors for predicting relapse by comparing initial clinical and laboratory findings.

Validation of the MCK model for predicting outcomes in myopathy-associated interstitial lung disease: a multicentre MYKO cohort study.

Objectives: To validate the MCK model's prognostic utility in patients with idiopathic inflammatory myopathy (IIM)-associated interstitial lung disease (ILD).

Methods: This retrospective study included 242 patients with IIM-associated ILD from the multicentre MYKO cohort. Patients were classified as anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive or antibody-negative.

Roles of JAK-STAT signaling and granulocyte-macrophage colony-stimulating factor in the development of osteitis and bone microstructure changes in rheumatoid arthritis.

Background: Osteitis on magnetic resonance imaging (MRI) and bone microstructure changes (BMC) on high-resolution peripheral quantitative computed tomography (CT) are the earliest signs of arthritis, preceding the development of bone erosion on X-ray in rheumatoid arthritis (RA). Recently, a Janus kinase (JAK) inhibitor, baricitinib, reportedly suppresses these early changes. This study aimed to elucidate the underlying molecular mechanism of osteitis and BMC using an animal model of RA and human samples.

Immune checkpoint inhibitor-induced myocarditis and multiple adverse events with pre-existing rheumatoid arthritis: a case report and literature review.

Immune checkpoint inhibitors (ICIs) can trigger immune-related adverse events (irAEs) and rheumatoid arthritis (RA) reactivation in cancer patients with pre-existing RA. Studies indicate RA reactivation occurs in approximately 50% of these patients, while new irAEs develop in 25-50% of ICI-treated patients. Furthermore, ICI-induced myocarditis has been reported to have a high mortality rate, ranging from 25% to 50%.

Differences in Organ Damage Based on Age at Onset in Idiopathic Inflammatory Myopathies: A Retrospective Multicenter MYKO Study.

Objectives To analyze the influence of age of the onset on myositis organ damage and to identify the factors influencing myositis organ damage, as clinical manifestations of myopathies differ by the age of onset and the background of patients. Methods Factors influencing organ damage [SLICC/ACR Damage Index (SDI)] were identified using the Japanese multicenter myositis registry (MYKO, n=220). Factors influencing organ damage were identified using a multivariate analysis.

Anti- Melanoma Differentiation-Associated Gene 5 Antibody Positive Dermatomyositis: Recent Progress in Pathophysiology and Treatment.

Purpose Of Review: Anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis (MDA5-DM) is a rare systemic autoimmune disease characterized by a clinically amyopathic presentation and a high-risk association with rapidly progressive interstitial lung disease. Although frequently fatal, the underlying mechanisms remain incompletely understood. This review provides a comprehensive summary of recent advances in research on MDA5-DM, aiming to deepen our understanding of its pathogenic mechanisms and to accelerate future basic research that will contribute to the development of novel therapeutic strategies.

Clinicopathological features of anti-HMGCR and anti-SRP myopathies that do not satisfy the EULAR/ACR criteria of inflammatory myopathies.

Objectives: The clinicopathological features of immune-mediated necrotizing myopathy (IMNM) sometimes mimic muscular dystrophy, complicating accurate diagnosis. The European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR) classification criteria of idiopathic inflammatory myopathies (IIMs) are superior in terms of sensitivity and specificity; however, the sensitivity is reported to be relatively low in IMNM. We examined the clinicopathological characteristics and the prognoses of anti-3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) antibody-positive and anti-signal recognition particle (SRP) antibody-positive cases that do not satisfy the EULAR/ACR classification criteria.

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.

Anti-arthritic effects of polyunsaturated fatty acid-rich supplementation combined with selective soluble epoxide hydrolase inhibitors in a collagen-induced arthritis mouse model.

Objectives: Polyunsaturated fatty acids (PUFAs) are healthy fats that contain a double carbon bond. They are made up of omega (ω)-3 and ω-6 PUFAs, where ω-3 PUFAs exhibit beneficial effects, such as the suppression of cardiovascular events and the improvement of abnormal lipid metabolism. However, their effects on rheumatoid arthritis (RA) are unclear.

Necrotising renal vasculitis associated with sarcoidosis in a patient with oesophageal cancer: A case report.

Sarcoidosis is a multisystem disorder characterised by noncaseating granulomas, often involving the lungs and lymph nodes, but can affect nearly any organ. Renal involvement in sarcoidosis typically presents as hypercalcaemia or interstitial granulomatous nephritis. Renal vasculitis, however, is an exceedingly rare manifestation.

The Association of Anti-dsDNA Antibodies with Patient-reported Outcomes of Patients with Systemic Lupus Erythematosus in a Two-consecutive Year Prospective Study.

Objectives: To analyse the association of anti-dsDNA Ab with patient-reported outcomes (PROs) in patients with SLE under maintenance treatment, as it has not been clearly understood whether PROs are able to be reflected by anti-dsDNA, which are associated with SLE activities (SLE Disease Activity Index 2000, SLEDAI-2K).

Methods: The SLE symptom checklist (SSC), LupusPRO, Medical Outcomes Study Short Form-36 (SF-36), and patient and physician visual analogue scale (Pt/Ph-VAS) at a time point were evaluated for correlation with anti-dsDNA using the Kyoto Lupus Cohort Registry (n = 310) from 2019 to 2020. Further, associations between changes in anti-dsDNA with those in Pt/Ph-VAS and SSC at two time points of short-term (3 months) or long-term (2 years) time points.

Memory B cells and their transcriptomic profiles associated with belimumab resistance in systemic lupus erythematosus in the maintenance phase.

The factors contributing to the treatment efficacy of belimumab in patients with systemic lupus erythematosus (SLE) in the maintenance phase are unknown. Here, we collected blood samples from patients with SLE (n=44) treated with belimumab before and three and six months after treatment. RNA-Seq of whole blood was performed, and gene expression was quantified.

Differences in the autoantibody phenotypes and long-term outcomes between juvenile- and adult-onset systemic sclerosis.

Objective: To investigate differences in autoantibodies, clinical features, and long-term outcomes between juvenile- and adult-onset systemic sclerosis (SSc).

Methods: Autoantibodies and survival rates over a maximum of 20 years were retrospectively analysed in 504 Japanese patients with SSc (juvenile-onset SSc, n = 17; adult-onset SSc, n = 487) using data from Kyoto University Registry.

Results: : The autoantibodies observed were anti-topoisomerase-I (71% vs.

Anti-interferon gamma-inducible protein 16 antibodies: Identification of a novel autoantigen in idiopathic interstitial pneumonia and its clinical characteristics based on a multicenter cohort study.

Autoantibodies are detected in idiopathic interstitial pneumonias (IIPs) without a clear connective tissue disease diagnosis, and their clinical significance is unclear. This study aimed to identify a novel autoantibody in IIPs. We screened 295 IIP patients using a S-methionine labeled protein immunoprecipitation assay.

Factors affecting discrepancies in disease activity evaluation between patients and physicians in systemic lupus erythematosus-The importance of symptoms such as fatigue.

Objectives: There are often discrepancies in the evaluation of disease activity between patients and physicians in systemic lupus erythematosus (SLE). In this study, we examined the factors that affect those evaluations.

Methods: Physician visual analogue scale (Ph-VAS), patient VAS (Pt-VAS), Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2k), glucocorticoid (GC) usage and dose, age, Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, and three patient-reported outcomes (SLE symptom checklist [SSC], short-form 36 questionnaire [SF-36], and LupusPRO) were obtained from a study performed in 2019 using 225 SLE outpatients of the Kyoto Lupus Cohort at Kyoto University Hospital.

A case of vesiculobullous dermatomyositis with anti-NXP-2 antibody without malignancy.

Vesiculobullous dermatomyositis (VD) is a rare manifestation of dermatomyositis (DM) and has been suggested to be associated with malignancy. Although the myositis-specific autoantibodies are associated with distinct clinical presentations of DM, those associated with VD remain unclear. Here, we present the case of a 54-year-old man with VD who tested positive for antinuclear matrix protein 2 (NXP-2) antibodies, one of the DM-specific autoantibodies.

[Treatment Strategies for Anti-Synthetase Syndrome].

Anti-aminoacyl tRNA synthetase (ARS) antibodies are the most frequent in idiopathic inflammatory myopathy, notably associated with anti-synthetase syndrome (ASyS), which is characterized by six clinical features: arthritis, myositis, interstitial lung disease (ILD), fever, Raynaud's phenomenon, and mechanical hands. Although patients with ASyS often respond well to initial glucocorticoid (GC) therapy, they tend to have a chronic, recurrent disease course. In anti-ARS-positive patients, the treatment goal involves suppressing disease recurrence and progression while achieving a minimal GC dose.

Risk factors for serious infections and infection-related mortality in patients with microscopic polyangiitis: Multicentre REVEAL cohort study.

Objective: Infections are a critical concern for patients with microscopic polyangiitis (MPA). This study aimed to identify the risk factors associated with serious infections (SIs) and infection-related mortality in patients with MPA, as well as the effect of glucocorticoid (GC) dose tapering on these outcomes.

Methods: This multicentre, retrospective, and observational study utilised data from a cohort of patients with MPA in Japan [Registry of Vasculitis Patients to Establish REAL World Evidence (REVEAL) cohort].