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A 51-year-old male patient presented with fever, myalgia, and bilateral thigh pain, followed by proximal weakness of limbs. Laboratory features were suggestive of inflammatory polymyositis (PM). He initially responded to steroids, but subsequently, the patient's clinical condition deteriorated. In the initial investigations, there was no evidence of any malignancy. Over the next 2 months, he had a catastrophic downhill course—progressive pancytopenia, acute abdominal pain, and features of mesenteric panniculitis and he eventually was diagnosed with acute myeloid leukemia (AML). This case underscores the high index of suspicion for underlying malignancy while managing cases of refractory PM and emphasizes the importance of comprehensive diagnostic evaluation and interdisciplinary collaboration in managing patients with complex autoimmune and hematological conditions. Jo-1 antibody, also known as anti-histidyl-tRNA synthetase antibody, is a specific type of autoantibody associated with a group of autoimmune diseases collectively known as idiopathic inflammatory myopathies (IIMs), particularly polymyositis (PM) and dermatomyositis (DM). Here, we present a case report highlighting the rare clinical association between Jo-1-positive PM and hematological malignancy. The presence of Jo-1 antibodies in this context may suggest potential immune dysregulation or paraneoplastic phenomena linking PM and AML. Accurate diagnosis and timely intervention are crucial for managing patients with complex autoimmune and hematological conditions, especially when they present with atypical clinical features and show poor response to treatment. [ABSTRACT FROM AUTHOR]

Objective: To identify the risk factors for relapse of idiopathic inflammatory myopathies (IIMs). Methods: Patients who were newly diagnosed with IIMs and underwent muscle biopsy between 2000 and 2017 at Asan Medical Center were retrospectively reviewed. The relapse of IIMs was defined as the recurrence of muscle or cutaneous manifestations with a ≥50% increase in glucocorticoid dosage after reaching the low-dose glucocorticoid phase with clinically significant improvement. The factors associated with the relapse of IIMs were investigated by Cox proportional hazards analysis. Results: Of 105 patients with IIMs, relapse was observed in 65 patients (62%). The titer of antinuclear antibody (ANA) was higher in the relapse group than in the non-relapse group (P = 0.033). Multivariable analysis showed that the relapse of IIMs was significantly associated with histopathologic features consistent with IIMs (hazard ratio [HR], 1.69; 95% confidence interval [CI], 1.01–2.83, P = 0.045) and the use of immunosuppressants before relapse (HR, 0.50; 95% CI, 0.29–0.86, P = 0.013). Doubling of ANA titer was also associated with relapse, albeit without statistical significance (HR, 1.13; 95% CI, 1.00–1.27, P = 0.052). Conclusion: In patients with IIMs, the use of immunosuppressants had a significant negative association with relapse. Administering immunosuppressants from the early period during the initial glucocorticoid tapering phase may be useful in reducing the risk of relapse in patients with IIMs. Key Points • Since idiopathic inflammatory myopathies (IIMs) have a low prevalence, it is poorly understood which factors are associated with the relapse of IIMs. • In this study, about two-thirds of 105 patients with IIMs experienced a relapse of IIMs. • The risk of relapse in patients with IIMs was negatively associated with the use of immunosuppressants during glucocorticoid tapering and low-dose glucocorticoid phase. • Even in less severe cases, the use of immunosuppressants might be a good option for the management of IIMs. [ABSTRACT FROM AUTHOR]

Background: The Myositis Interstitial Lung Disease Nintedanib Trial (MINT) is a hybrid trial, which is enrolling patients both at local sites and remotely via a decentralised site. The trial will investigate the efficacy and safety of nintedanib in patients with progressive myositis-associated interstitial lung disease (MA-ILD). Methods/Design: MINT is an exploratory, prospective randomised placebo-controlled trial. Eligible patients will have myositis and evidence of fibrosing ILD on high-resolution computed tomography (HRCT), be taking standard of care medications for myositis, and meet criteria for ILD progression within the prior 24 months based on decline in FVC, worsened fibrosis on HRCT, and/or worsened dyspnoea. Patients will be randomised 1:1 to receive nintedanib 150 mg twice daily or placebo for 12 weeks then open-label nintedanib for 12 weeks. Patients will be enrolled at local sites and a decentralised site. Most study visits will be completed remotely using telemedicine or digital health technologies. The primary endpoint is the change in Living with Pulmonary Fibrosis (L-PF) questionnaire dyspnoea domain score at week 12. Other endpoints include changes in other L-PF questionnaire domains, lung function, imaging, and physical activity, and assessment of adverse events. Data collected using remote versus clinic enrolment, and using home versus clinic spirometry, will be compared. Discussion: MINT is an innovative, hybrid trial that will evaluate the effects of nintedanib on symptoms, quality of life, and ILD progression in patients with progressive MA-ILD and provide valuable information on the utility of decentralised recruitment and remote data collection in clinical trials. Trial registration: Clinicaltrials.gov NCT05799755 (date of registration: 05/04/2023). [ABSTRACT FROM AUTHOR]

Background: the reference method for detection of myositis-specific and myositis-associated antibodies (MSAs and MAAs) is considered immunoprecipitation (IP), but it is routinely replaced by semi-automated methods, like lineblot (LB). Few data are available on the consistency with clinical diagnoses; thus, we aim at analysing these aspects. Methods: sixty-nine patients with idiopathic inflammatory myopathies (IIM) were studied via LB (Myositis Antigens Profile 3 EUROLINE, Euroimmun) and IP (RNA and protein antigens). The degree of concordance between methods was calculated using Cohen's coefficient. Results: a substantial concordance was found for anti-Ku and anti-PM/Scl and a moderate concordance was found for anti-Jo1 and anti–Mi-2, while a fair concordance was found for anti-EJ, anti-SRP, and anti-Ro52 antibodies. The concordance could not be calculated for anti-OJ, anti-PL-7, anti-PL-12, anti-NXP2, anti-TIF1ɣ, and anti-MDA5, because they were only detected with one method. Multiple MSAs were found only with LB in 2/69 sera. Anti-MDA5, TIF1ɣ, NXP2 (detected via IP), and anti-Jo1 in anti-synthetase syndrome (both LB and IP) had the best concordance with clinical diagnosis. Conclusions: LB and IP show substantial concordance for PM/Scl and Ku, and moderate concordance for Jo1 and Mi-2, with a good concordance with clinical diagnoses. IP shows a high performance for DM-associated MSAs. LB seems to be more sensitive in detecting anti-Ro52 antibodies, but it identified multiple MSAs, unlike IP. [ABSTRACT FROM AUTHOR]

Objective Idiopathic inflammatory myopathies (IIMs) are autoimmune disorders significantly impacting skeletal muscles; however, the precise correlation between muscle magnetic resonance imaging (MRI) findings, muscle pathology, disease subtypes and clinical characteristics remains uncertain. Thus, we investigated the association of muscle MRI findings in IIMs with muscle pathology and clinical features. Methods New-onset IIM patients underwent proximal upper and/or lower limb muscle MRI. Patterns of muscle oedema on MRI were categorised into fascial, honeycomb, peripheral, foggy, dense, or coarse dot patterns and compared with inflammatory cell infiltration sites in corresponding muscle biopsies. The incidence of MRI patterns was examined in patient subgroups using myositis-specific antibodies (MSAs) and 2017 EULAR/ACR classification criteria. Univariate and multivariate analyses were conducted to determine the odds ratios (ORs) of MRI findings for clinical characteristics. Results Fifty-six of 85 patients underwent muscle biopsy. Foggy, honeycomb and fascial patterns at biopsy sites correlated with inflammatory cell infiltration in the endomysium (OR 11.9, P  = 0.005), perimysium (OR 6.0, P  = 0.014) and fascia (OR 16.9, P  < 0.001), respectively. Honeycomb and foggy patterns were characteristic of patients with anti-TIF1γ or anti-Mi2 antibodies and MSA-negative dermatomyositis, and those with anti-SRP or anti-HMGCR antibodies and MSA-negative polymyositis (PM), respectively. The honeycomb pattern positively correlated with malignancy (OR 6.87, P  < 0.001) and Gottron sign (OR 8.05, P  = 0.002); the foggy pattern correlated with muscle weakness (OR 11.24, P  = 0.005). The dense dot pattern was associated with dysphagia (OR 6.27, P  = 0.006) and malignancy (OR 8.49, P  = 0.002). Conclusion Muscle MRI holds promise in predicting muscle pathology, disease subtypes and clinical manifestations of IIMs. [ABSTRACT FROM AUTHOR]

Objective: Idiopathic inflammatory myopathies (IIM) confer an increased risk of morbidity from atherosclerotic cardiovascular disease (ASCVD). While ASCVD risk has been studied in other countries, these results may not be applicable to patients with dermatomyositis (DM) and polymyositis (PM) in the United States. This retrospective analysis of a cohort of patients identified by ICD code from TriNetX investigated the incidence of ASCVD after International Classification of Disease (ICD) codes of DM, PM, dermatopolymyositis (DPM) or juvenile dermatomyositis (JDM). Method: Patients were identified by entry of two ICD codes separated by at least 6 months, according to their first diagnosis code; ASCVD was defined as first ICD code for myocardial infarction, ischemic stroke, transient ischemic attack, or peripheral arterial disease. Cox proportional hazards regression modeled time from first IIM ICD code to ASCVD event. Results: A total of 35,554 patients were identified with the mean age at first IIM code of 54 and 26.1% were male. The most common comorbidity for all groups except JDM was hyperlipidemia (39.9%) though 79.2% of patients were on no cholesterol lowering medication. ASCVD occurred in 30.4% of patients with PM, 24.3% of patients with DM and 0.9% of patients with JDM. Patients with PM had a median time to event of 9.7 years (95% Confidence interval (CI) 9.1, 10.7) and 14.3 years (95% CI 12.6, 14.8) for DM. This study demonstrates that ASCVD is a comorbidity occurring after a median of 12.5 years (95% CI 11.9, 13.6) in patients with IIM. Conclusions: ASCVD appears to be a long-term complication for IIM patients occurring in nearly a quarter of US patients without prior ASCVD with at least two ICD codes for IIM, with a median time to event of 12.5 years. There appears to be a practice gap in the recognition and treatment of hyperlipidemia in these patients. Key Points • Hyperlipidemia was a common comorbidity identified in patients with IIM though most patients were not on cholesterol lowering medication. • Development of ASCVD appears to be a long-term complication for patients with IIM in the United States. [ABSTRACT FROM AUTHOR]

Inflammatory myopathies, such as polymyositis and dermatomyositis, are systemic inflammatory disorders that affect skeletal muscles and internal organs. The treatment of inflammatory myopathies usually involves long-term use of high doses of steroids and/or immunosuppressants, making patients susceptible to opportunistic infections. Unfortunately, infections are a leading cause of morbidity and mortality in patients with inflammatory myopathies. Musculoskeletal nontuberculous mycobacterial infections are rare. Nontuberculous mycobacterial infections are easily overlooked owing to their rarity, leading to delayed diagnosis and treatment, indolent clinical course, and difficulty isolating the pathogen. Nontuberculous mycobacterial infections are a growing health concern because of their increasing incidence and the need for prolonged treatment. In patients with connective tissue diseases, immunosuppressant use may lead to an increased risk of nontuberculous mycobacterial infection with a poor prognosis, which highlights the need for early diagnosis and treatment. Herein, we report the case of a 59-year-old man diagnosed with dermatomyositis, who had prolonged use of immunosuppressants and developed a disseminated soft tissue infection in both thighs caused by Mycobacterium abscessus. Multimodal images were obtained using magnetic resonance imaging, ultrasonography, and computed tomography. A strong suspicion of possible combined opportunistic infections and appropriate staining is essential in diagnosing nontuberculous mycobacterial myositis. [ABSTRACT FROM AUTHOR]

Introduction: Sjogren's syndrome (SS) is an autoimmune chronic inflammatory disorder affecting women in their fourth to sixth decade, affecting gastrointestinal, and musculoskeletal systems. A 35-year-old female with SS with polymyositis (PM) presented with symptoms of weakness in all four limbs, difficulty in sitting, rising, swallowing solid foods, vomiting, and difficulty in climbing stairs. She was diagnosed with SS in 2018 and was treated with prednisolone, Vitamin D, calcium, pyridoxine, methylcobalamin, artificial tears, pilocarpine, and painkillers. However, a muscle biopsy was never done. Methods: The data were collected from the patient's file along with her consent when she came for follow-up. Results: A muscle biopsy was done at our center to confirm the diagnosis of PM. The patient was prescribed IV prednisolone and other symptomatic treatment until symptoms resolved and was discharged with oral drugs when they were manageable. Conclusion: The diagnosis of primary SS along with PM is a rare occurrence. Although it does not change the treatment plan much, its diagnosis is very important for managing any complications that may arise from it. Patients' noncompliance and loss of follow-up can create issues in the treatment. Such cases help in forming the guidelines for the future and restructuring the classification of autoimmune conditions. [ABSTRACT FROM AUTHOR]

Objective: Observational studies have revealed a higher probability of hypothyroidism in patients with dermatomyositis (DM) or polymyositis (PM), but there is no consensus on whether hypothyroidism causally influences DM or PM. In the present study, we assessed the causal association between hypothyroidism and the risk of dermatomyositis or polymyositis using two-sample Mendelian randomization (TSMR). Methods: The genome-wide association data of hypothyroidism and dermatomyositis/polymyositis were obtained from the IEU Open GWAS project. Then, TSMR was used to determine whether hypothyroidism is causally associated with DM or PM. Single-nucleotide polymorphisms (SNPs) significantly associated with hypothyroidism were identified and used as instrumental variables (IVs), and the causal relationship between hypothyroidism and DM/PM was examined using TSMR. MR pleiotropy and Cochran's Q test were used to confirm the heterogeneity and pleiotropy of identified IVs, then four different models, including the inverse variance weighted model (IVW), MR-Egger, weighted median and weighted model were applied in this MR analysis. Results: Sixty-eight SNPs for DM and 68 SNPs for PM were selected as the IVs (P<5x10-8; linkage disequilibrium R² <0.001) to assess the causal association between hypothyroidism and DM/PM selected from GWASs on hypothyroidism. The results revealed a positive causal effect of hypothyroidism on both DM and PM (DM: OR 2.563, 95% CI [1.348, 4.874], P = 0.00156; PM: OR1.709, 95% CI [1.157, 2.525], P =0.007). Moreover, there was no heterogeneity or pleiotropy in the results. Conclusion: In conclusion, the MR analysis results provided strong evidence to indicate that hypothyroidism might be causally associated with DM and PM. These findings may have important implications for the pathogenesis and possible future therapies of DM/PM. [ABSTRACT FROM AUTHOR]

Background/Objectives: Idiopathic inflammatory myopathies (IIMs) are rare autoimmune disorders characterized by progressive proximal muscle weakness and varying extra-muscular manifestations. The latest 2017 EULAR/ACR criteria classify them into subgroups. This study aims to evaluate the role of nailfold capillaroscopy (NFC) as a diagnostic and prognostic tool in IIMs by comparing capillaroscopic patterns across different IIM subtypes. Methods: We conducted an observational, cross-sectional study at the Institute of Rheumatology in Belgrade, analyzing 90 patients diagnosed with IIMs per the 2017 EULAR/ACR criteria. Patients were categorized into dermatomyositis (DM) (n = 37), polymyositis (PM) (n = 35), amyopathic dermatomyositis (ADM) (n = 13), and juvenile dermatomyositis (JDM) (n = 5). A control group of 35 patients with primary Raynaud's phenomenon was also included. NFC findings, clinical manifestations, and laboratory data were compared across the groups. Results: In DM, 81.9% exhibited a scleroderma capillaroscopic pattern, which was also present in 76.9% of ADM patients. In PM, the most common pattern was nonspecific changes (48.6%). JDM patients showed a high prevalence of scleroderma changes (n = 4 (80%)). Scleroderma patterns correlated with Gottron's papules, heliotrope rash, periungual erythema, Raynaud's phenomenon, and interstitial lung disease (ILD). No significant differences were found in laboratory parameters across capillaroscopic groups, except for a higher prevalence of anti-Jo1 antibodies in patients with nonspecific capillaroscopic changes. Conclusions: NFC is a valuable tool for differentiating IIM subtypes and correlating clinical manifestations with specific capillaroscopic patterns. The high prevalence of scleroderma changes in DM and ADM suggests their potential as a diagnostic and prognostic marker in IIMs. Further research with larger cohorts is warranted to validate these findings. [ABSTRACT FROM AUTHOR]

Idiopathic inflammatory myopathies (IIMs) encompass a spectrum of autoimmune diseases characterized by muscle inflammation and systemic involvement. This review aimed to synthesize current evidence on the clinical significance and pathogenic mechanisms underlying autoantibodies associated with IIMs. Autoantibodies targeting aminoacyl-tRNA synthetases (ARS) play a pivotal role in antisynthetase syndrome (ASS), highlighting associations with interstitial lung disease (ILD) and distinctive clinical features. Anti-Mi-2 antibodies in dermatomyositis (DM) are hallmarked by characteristic cutaneous manifestations and favorable prognostic outcomes. Conversely, anti-TIF1 antibodies are correlated with DM and a higher risk of malignancies, implicating CD8+ T cells in its pathogenesis. Anti-MDA5 antibodies signify clinically amyopathic DM (CADM) with severe ILD, linked to dysregulated neutrophil extracellular trap (NET) formation. In immune-mediated necrotizing myopathies (IMNMs), anti-SRP and anti-HMGCR antibodies induce complement-mediated myopathy, typically following statin exposure. Additionally, anti-TRIM72 antibodies emerge as potential diagnostic markers in IIMs. Anti-cN1A autoantibodies are linked to inclusion body myositis (IBM) and play a decisive role in muscle protein degradation. Meanwhile, anti-FHL1 autoantibodies are associated with severe disease manifestations and muscle damage, as established in experimental models. Anti-eIF3 autoantibodies, recently identified in polymyositis (PM) patients, are rarely detected (<1%) and associated with a favorable prognosis. Elucidating these autoantibodies is anticipated to not only assist in early diagnosis and disease stratification but also inform targeted therapeutic interventions, emphasizing the intricate interplay between autoimmunity, cellular dysfunction, and clinical outcomes in IIMs. [ABSTRACT FROM AUTHOR]

The epidemiology of idiopathic inflammatory myopathies (IIMs) varies by country. Investigating the epidemiological profile among Thai IIMs could help to inform public health policy, potentially leading to cost-reducing strategies. We aimed to assess the prevalence and incidence of IIM in the Thai population between 2017 and 2020. A descriptive epidemiological study was conducted on patients 18 or older, using data from the Information and Communication Technology Center, Ministry of Public Health, with a primary diagnosis of dermatopolymyositis, as indicated by the ICD-10 codes M33. The prevalence and incidence of IIMs were analyzed with their 95% confidence intervals (CIs) and then categorized by sex and region. In 2017, the IIM cases numbered 9,074 among 65,204,797 Thais, resulting in a prevalence of 13.9 per 100,000 population (95% CI 13.6–14.2). IIMs were slightly more prevalent among women than men (16.8 vs 10.9 per 100,000). Between 2018 and 2020, the incidence of IIMs slightly declined from 5.09 (95% CI 4.92–5.27) in 2017 and 4.92 (95% CI 4.76–5.10) in 2019 to 4.43 (95% CI 4.27–4.60) per 100,000 person-years in 2020. The peak age group was 50–69 years. Between 2018 and 2020, the majority of cases occurred in southern Thailand, with incidence rates of 7.60, 8.34, and 8.74 per 100,000 person-years. IIMs are uncommon among Thais, with a peak incidence in individuals between 60 and 69, especially in southern Thailand. The incidence of IIMs decreased between 2019 and 2020, most likely due to the COVID-19 pandemic, which reduced reports and investigations. [ABSTRACT FROM AUTHOR]

Introduction: Observational studies suggest associations between gut microbiota and polymyositis (PM) and dermatomyositis (DM), but causal relationships are unclear. We investigate the causal effects of gut microbiota on PM and DM, providing insights hoping to provide insights for future treatment and prevention. Methods: Summary statistics of gut microbiota were obtained from a multi-ethnic Genome Wide Association Studies (GWAS) meta-analysis, including 119 taxa from 18,340 Europeans. PM/DM statistics were extracted from GWAS analyses. Mendelian randomization (MR) with IVW, MR-Egger, and weighted median methods was performed. Sensitivity analyses addressed heterogeneity and pleiotropy. Of the 119 bacterial genera studied, six showed causal links. Results: Alloprevotella (OR: 3.075, 95% CI: 1.127-8.386, p = 0.028), Ruminococcaceae UCG003 (OR: 4.219, 95% CI: 1.227-14.511, p = 0.022), Dialister (OR: 0.273, 95% CI: 0.077-0.974, p = 0.045) were associated with PM. Anaerotruncus (OR: 0.314, 95% CI: 0.112-0.882, p = 0.028), Ruminococcaceae UCG002 (OR: 2.439, 95% CI: 1.173-5.071, p = 0.017), Sutterella (OR: 3.392, 95% CI: 1.302-8.839, p = 0.012) were related to DM. Sensitivity analyses validated these associations. Discussion: We establish causal relationships between Ruminococcaceae, Sutterella, Anaerotruncus with DM, Alloprevotella, Ruminococcaceae UCG003, and Dialister with PM. Common microbiota, like Ruminococcaceae, have significant clinical implications. These findings open up greater possibilities for the gut microbiota to contribute to the development of PM/DM and for future monitoring of the gut microbiota in patients with PM/DM. [ABSTRACT FROM AUTHOR]

Objectives To assess the ability of dual-energy X-ray absorptiometry (DXA) and hand-grip dynamometer to measure damage in inflammatory myopathies (IM). Methods Forty adult IM patients with a disease duration ≥12 months, low or no disease activity for ≥6 months, were prospectively enrolled. Thirty healthy age and sex-matched volunteers were enrolled as controls. Whole-body DXA and hand-grip dynamometer were used to measure muscle mass, grip strength and diagnose sarcopenia (EWGSOP2 criteria). Relationships between the results of strength in 12 muscles, functional tests, patient-reported disability, IMACS damage score, and history of the disease were assessed. The serum levels of potential molecular actors in the damage were measured. Results DXA and grip strength measurements took ≤20 min. Both muscle mass and grip strength were decreased in IM patients vs volunteers (−10% and −30%, respectively) with a dispersion that varied widely (interquartile range −24.3% to +7.8% and −51.3% to −18.9%, respectively). Muscle mass and grip strength were non-redundantly correlated (r up to 0.6, P  = 0.0001) with strength in 14 muscles (manual muscle test and hand-held dynamometer), functions (of limbs, respiratory and deglutition muscles), patient-reported disability, damage (extension and severity in muscular and extra-muscular domains) and blood levels of several myokines. Seven IM patients (17.5%) were sarcopenic. They had the worst damage, impaired functions, disability and history of severe myopathy. Decreased irisin and osteonectin levels were associated with sarcopenia (area under the curve 0.71 and 0.80, respectively). Conclusion DXA and hand-grip dynamometer are useful tools to assess damage in IM. Irisin and osteonectin may play a role in IM damage pathogenesis. [ABSTRACT FROM AUTHOR]

Objectives Myositis-specific and associated autoantibodies are important biomarkers in routine clinical use. We assessed local testing performance for myositis autoantibodies by comparing line immunoassay (LIA) to protein radio-immunoprecipitation and identifying clinical characteristics associated with each myositis autoantibody in the MyoCite cohort. Methods Serum samples from patients within the MyoCite cohort, a well-characterized retro-prospective dataset of adult and juvenile idiopathic inflammatory myopathy (IIM) patients in Lucknow, India (2017–2020), underwent LIA at Sanjay Gandhi Postgraduate Institute of Medical Science (SGPGIMS), Lucknow. Immunoprecipitation of 147 IIM patients' serum samples (125 adult-onset, 22 juvenile-onset) was conducted at the University of Bath, with researchers blind to LIA results. LIA performance was assessed against immunoprecipitation as the reference standard, measuring sensitivity, specificity and inter-rater agreement. Univariate and multivariate logistic regression determined clinical associations for specific myositis-specific autoantibodies. Results Immunoprecipitation identified myositis autoantibodies in 56.5% (n  = 83) of patient samples, with anti-Jo1 (n  = 16; 10.9%) as the most common, followed by anti-MDA5 (n  = 14, 9.5%). While LIA showed good agreement for anti-Jo1, anti-PL7 and anti-PL12 (Cohen's κ 0.79, 0.83 and 1, respectively), poor agreement was observed in other subgroups, notably anti-TIF1γ (Cohen's κ 0.21). Strongly positive samples, especially in myositis-specific autoantibodies, correlated more with immunoprecipitation results. Overall, 59 (40.1%) samples exhibited non-congruence on LIA and immunoprecipitation, and κ values for LIAs for anti-TIF1γ, anti-Ku, anti-PmScl, anti-Mi2 and anti-SAE ranged between 0.21 and 0.60. Conclusion While LIA reliably detected anti-Jo1, anti-PL7, anti-PL12, anti-MDA5 and anti-NXP-2, it also displayed false positives and negatives. Its effectiveness in detecting other autoantibodies, such as anti-TIF1γ, was poor. [ABSTRACT FROM AUTHOR]

Objective: This study investigated the prognostic factors of anti-melanoma differentiation-associated gene 5 antibody-positive dermatomyositis (MDA5-DM). Methods: This study analysed 34 MDA5-DM cases (20 and 14 in the survival and death groups, respectively) encountered at Kurume University between 2008 and 2021. The clinical, physiological, and computed tomography findings, pulmonary function, and serological results were retrospectively evaluated for each MDA5-DM case during the first visit and throughout the next 12 weeks. Results: In the death group, the mean age of patients was higher (47.6 vs. 61.8 years), while the duration from symptom onset to consultation was shorter (110 vs. 34.9 days). During the first visit, the death group demonstrated a significantly higher serum C-reactive protein level (0.52 vs. 1.99) and a significantly lower albumin level (3.23 vs. 2.63) than the survival group; this persisted throughout the next 12 weeks. Poor prognosis was associated with C-reactive protein and albumin levels >0.19 mg/dl and <2.3 g/dl, respectively, 4 weeks after starting the treatment. Conclusion: Four weeks after starting the treatment, serum C-reactive protein and albumin levels of patients with MDA5-DM can be used to evaluate treatment response and predict prognosis. [ABSTRACT FROM AUTHOR]

Polymyositis (PM) and dermatomyositis (DM) are chronic inflammatory muscle disorders characterized by muscle weakness and fatigue. They are histopathologically defined by inflammatory cell infiltrates in the skeletal muscle. In a recent meta-analysis, the incidence of cardiovascular complications in patients with PM/DM ranged from 9 to 72 %, with heart failure being the most commonly reported heart disease. In cases of heart failure accompanied by severe mitral regurgitation that is refractory due to guideline-directed medical therapy, the transcatheter edge-to-edge mitral valve repair (TEER) procedure using the MitraClip (Abbott Vascular, Santa Clara, CA, USA) offers an alternative therapeutic approach. We present a pioneering case report describing the successful application of TEER in a young woman with drug-refractory heart failure due to cardiovascular involvement in PM. • In polymyositis (PM)/ dermatomyositis (DM), the most commonly reported clinical heart disorder is heart failure, with some cases attributed to mitral regurgitation. • Patients suffering from systemic inflammatory diseases such as PM/DM often face high surgical risk, and the MitraClip is an effective treatment modality. [ABSTRACT FROM AUTHOR]

Abstract A 51-year-old male patient presented with fever, myalgia, and bilateral thigh pain, followed by proximal weakness of limbs. Laboratory features were suggestive of inflammatory polymyositis (PM). He initially responded to steroids, but subsequently, the patient’s clinical condition deteriorated. In the initial investigations, there was no evidence of any malignancy. Over the next 2 months, he had a catastrophic downhill course—progressive pancytopenia, acute abdominal pain, and features of mesenteric panniculitis and he eventually was diagnosed with acute myeloid leukemia (AML). This case underscores the high index of suspicion for underlying malignancy while managing cases of refractory PM and emphasizes the importance of comprehensive diagnostic evaluation and interdisciplinary collaboration in managing patients with complex autoimmune and hematological conditions. Jo-1 antibody, also known as anti-histidyl-tRNA synthetase antibody, is a specific type of autoantibody associated with a group of autoimmune diseases collectively known as idiopathic inflammatory myopathies (IIMs), particularly polymyositis (PM) and dermatomyositis (DM). Here, we present a case report highlighting the rare clinical association between Jo-1-positive PM and hematological malignancy. The presence of Jo-1 antibodies in this context may suggest potential immune dysregulation or paraneoplastic phenomena linking PM and AML. Accurate diagnosis and timely intervention are crucial for managing patients with complex autoimmune and hematological conditions, especially when they present with atypical clinical features and show poor response to treatment.

Abstract Background The Myositis Interstitial Lung Disease Nintedanib Trial (MINT) is a hybrid trial, which is enrolling patients both at local sites and remotely via a decentralised site. The trial will investigate the efficacy and safety of nintedanib in patients with progressive myositis-associated interstitial lung disease (MA-ILD). Methods/Design MINT is an exploratory, prospective randomised placebo-controlled trial. Eligible patients will have myositis and evidence of fibrosing ILD on high-resolution computed tomography (HRCT), be taking standard of care medications for myositis, and meet criteria for ILD progression within the prior 24 months based on decline in FVC, worsened fibrosis on HRCT, and/or worsened dyspnoea. Patients will be randomised 1:1 to receive nintedanib 150 mg twice daily or placebo for 12 weeks then open-label nintedanib for 12 weeks. Patients will be enrolled at local sites and a decentralised site. Most study visits will be completed remotely using telemedicine or digital health technologies. The primary endpoint is the change in Living with Pulmonary Fibrosis (L-PF) questionnaire dyspnoea domain score at week 12. Other endpoints include changes in other L-PF questionnaire domains, lung function, imaging, and physical activity, and assessment of adverse events. Data collected using remote versus clinic enrolment, and using home versus clinic spirometry, will be compared. Discussion MINT is an innovative, hybrid trial that will evaluate the effects of nintedanib on symptoms, quality of life, and ILD progression in patients with progressive MA-ILD and provide valuable information on the utility of decentralised recruitment and remote data collection in clinical trials. Trial registration Clinicaltrials.gov NCT05799755 (date of registration: 05/04/2023).

Myocarditis is a potentially life-threatening inflammatory disease of the myocardium, often resulting from infectious and immune-mediated responses. Clinical presentation in severe cases often results in a devastating illness requiring extracorporeal membrane oxygenation support as a result of cardiogenic shock. Although endomyocardial biopsy is still considered the gold standard for diagnosis, it often reveals nonspecific lymphocytic infiltration. Because the precise cause is usually unknown, the initial treatment typically involves immunosuppression and frequent assessment of myocardial contractility. This report presents 3 rare cases of autoimmune diseases (polymyositis, immunoglobulin G4–related disease, and systemic lupus erythematosus) that require extracorporeal membrane oxygenation support as a result of fulminant myocarditis, including their follow-up periods.

Abstract The epidemiology of idiopathic inflammatory myopathies (IIMs) varies by country. Investigating the epidemiological profile among Thai IIMs could help to inform public health policy, potentially leading to cost-reducing strategies. We aimed to assess the prevalence and incidence of IIM in the Thai population between 2017 and 2020. A descriptive epidemiological study was conducted on patients 18 or older, using data from the Information and Communication Technology Center, Ministry of Public Health, with a primary diagnosis of dermatopolymyositis, as indicated by the ICD-10 codes M33. The prevalence and incidence of IIMs were analyzed with their 95% confidence intervals (CIs) and then categorized by sex and region. In 2017, the IIM cases numbered 9,074 among 65,204,797 Thais, resulting in a prevalence of 13.9 per 100,000 population (95% CI 13.6–14.2). IIMs were slightly more prevalent among women than men (16.8 vs 10.9 per 100,000). Between 2018 and 2020, the incidence of IIMs slightly declined from 5.09 (95% CI 4.92–5.27) in 2017 and 4.92 (95% CI 4.76–5.10) in 2019 to 4.43 (95% CI 4.27–4.60) per 100,000 person-years in 2020. The peak age group was 50–69 years. Between 2018 and 2020, the majority of cases occurred in southern Thailand, with incidence rates of 7.60, 8.34, and 8.74 per 100,000 person-years. IIMs are uncommon among Thais, with a peak incidence in individuals between 60 and 69, especially in southern Thailand. The incidence of IIMs decreased between 2019 and 2020, most likely due to the COVID-19 pandemic, which reduced reports and investigations.

Antibodies to Severe Acute Respiratory Syndrome-Coronavirus 2 (SARS-CoV-2) have been reported in pooled healthy donor plasma and intravenous immunoglobulin products (IVIG). It is not known whether administration of IVIG increases circulating anti-SARS-CoV-2 antibodies (COVID ab) in IVIG recipients. COVID ab against the receptor binding domain of the spike protein were analyzed using a chemiluminescent microparticle immunoassay in patients with idiopathic inflammatory myopathies (IIM) both receiving and not receiving IVIG (IVIG and non-IVIG group, respectively). No significant differences in COVID ab levels were noted between IVIG and non-IVIG groups (417 [67-1342] AU/mL in IVIG vs 5086 [43-40,442] AU/mL in non-IVIG, p = 0.11). In linear regression models including all post-vaccination patient samples, higher number of vaccine doses was strongly associated with higher COVID ab levels (2.85 [1.21, 4.48] log AU/mL, regression coefficient [Formula: see text] [95% CI], p = 0.001), while use of RTX was associated with lower ab levels (2.73 [- 4.53, - 0.93] log AU/mL, [Formula: see text][95%CI], p = 0.004). In the IVIG group, higher total monthly doses of IVIG were associated with slightly higher COVID ab levels (0.02 [0.002-0.05] log AU/mL, p = 0.04). While patients on IVIG did not have higher COVID ab levels compared to the non-IVIG group, higher monthly doses of IVIG were associated with higher circulating levels of COVID ab in patients receiving IVIG, particularly in patients concomitantly receiving RTX. Our findings suggest that IIM patients, especially those at increased risk of COVID infection and worse COVID outcomes due to RTX therapy may have protective benefits when on concurrent IVIG treatment.

Abstract Background COVID-19 can induce a systemic inflammatory response with variable clinical manifestations. Similar to various viruses, COVID-19 has been implicated in the pathogenesis of autoimmune diseases. This article highlights the potential for infections including the SARS-CoV-2 virus to induce exacerbations of pre-existing autoimmune diseases or even potentially unmask de novo autoimmune diseases in particular anti-synthetase syndrome (ASSD) in predisposed individuals. Although there are other case reports of ASSD following SARS-CoV-2 infection, here we present the first reported case of a gentleman with a newly diagnosed anti-OJ positive anti-synthetase syndrome following SARS-CoV-2 infection. Case presentation Described is a case of a 70-year-old man presenting to the emergency department with worsening dyspnea in the context of a recent COVID-19 infection. CT-chest revealed changes suggestive of fibrotic lung disease, consistent with usual interstitial pneumonitis (UIP) pattern. Despite recovery from his COVID-19 illness, the patient subsequently developed proximal myopathy with cervical flexion weakness on further assessment with persistently elevated creatinine kinase (CK). Myositis autoantibodies found a strongly positive anti-OJ autoantibody with MRI-STIR and muscle biopsy performed to further confirm the diagnosis. The patient received pulse methylprednisolone 1 g for 3 days with a long oral prednisolone wean and in view of multiple end-organ manifestations, loading immunoglobulin at 2 g/kg administered over two days was given. In addition, he was then commenced and escalated to a full dose of azathioprine given a normal purine metabolism where he remains in clinical remission to this date. At least 267 cases of rheumatic diseases has been associated with SARS-CoV-2 infection as well as COVID-19 vaccination. A literature search on PubMed was made to determine the amount of case reports describing myositis associated with SARS-CoV-2 infection. We found 3 case reports that fit into our inclusion criteria. Further literature searches on diagnostic approach and treatment of ASSD were done. Conclusion Although SARS-CoV-2 infection itself can cause a directly mediated viral myositis, this case report highlights the possibility of developing virus-triggered inflammatory myositis through multiple aforementioned proposed mechanisms. Therefore, further studies are required to explore the relationship and pathophysiology of SARS-CoV-2 infection and the incidence of inflammatory myopathies.

Objective: The increasing use of the internet has resulted in its gaining a more active role in both patient and professional education, including the YouTube platform, a popular platform for sharing and watching videos. This study aimed to evaluate the quality, usefulness, and reliability of videos on inflammatory muscle diseases on YouTube, as well as their determinants for both patient and healthcare professional education. Materials and Methods: The keywords “Inflammatory muscle disease”, “Idiopathic inflammatory myositis”, “Inflammatory muscle disease treatment”, “Inflammatory myositis treatment”, “Dermatomyositis” and “Polymyositis” were searched on the site on April 22nd, 2022. According to the Global Quality scale, three categories (high quality, middle quality, and low quality) were created based on the educational value of YouTube videos. The reliability of the information was assessed using the DISCERN tool. The video parameters were compared between the quality groups to reveal the determinants of the quality. Results: Seventy-five videos of patients and 116 videos of healthcare professionals were included in the video after exclusion. The numbers of high, intermediate and low-quality videos were 27 (36%), 27 (36%), and 21 (28%) for videos for patients, and 20 (17.2%), 60 (51.8%), and 36 (31%), respectively. Only the number of likes was found to be a determinant of video quality for healthcare professionals (p0.05). Conclusion: YouTube is an open platform for information, and it includes various educational videos of various quality. Experts, such as physicians, should be encouraged to provide more content to help both patients and healthcare professionals obtain better quality information.

Background Inflammatory idiopathic myopathies (IIM) are a heterogeneous group of complex connective tissue diseases (CTD), primarily characterized by inflammatory involvement of skeletal muscles. Several other organs may also be affected, including lungs, heart, skin, gastrointestinal tract, and joints. Objective Describe the clinical and immunological characteristics of IIM among Tunisian patients. Method A retrospective study conducted in the internal medicine department at the Rabta Hospital over 22 years, including adult patients with IIM according to the ACR/EULAR classification criteria, and anti-synthetase syndrome (ASS) according to Connors’ criteria. Inclusion body myositis was excluded. Demographic, clinical, and immunological characteristics were analyzed. Results Ninety-seven patients were included (Male/female ratio= 0.36, mean age = 48.4 +- 13.8 years). The ACR/EULAR criteria enabled the inclusion of 88% of the patients. Dermatomyositis (DM) was the most frequent entity (47%), followed by Polymyositis (PM) (36%) and Amyotrophic dermatomyositis (ADM) (4%). Connors criteria allowed the inclusion and classification with ASS of 33 patients, including those excluded by the ACR/EULAR criteria (12%). Muscular involvement was present in 88% of patients, affecting locomotor (88%), gastrointestinal (43%), laryngeal (10%), cardiac (8%), and respiratory (1%) muscles. Myolysis was observed in 77% of patients, and histological evidence of myositis in 73%. ILD was reported across all subgroup: ASS (85%), PM (57%), DM (17%), and ADM (100%). NSIP was the most common radiologic pattern (59%). Skin involvement (85%), consistently present in DM and ADM, affected 91% of ASS patients. Joint involvement (48%) was predominantly polyarticular (68%). Myositis-specific antibodies (MSAs) were detected in 52% of patients, with a predominance of anti-synthetase antibodies (ASAs). Another CTD was noted in 24% of patients and cancer in 17% of patients. Conclusion Our study underscored the clinical diversity among IIM patients and the systemic nature of these diseases, where muscular involvement is not always uniform.

This study investigates the association between inflammatory myopathies (IM), and their correlation with cancer. There are several potential causes behind the association of cancer and inflammatory myopathies. The positivity of specific antibodies for myositis plays a significant role. Our objective is to describe cancer and inflammatory myopathies in Colombia, focusing on demographics, clinical characteristics, and laboratory data. We retrospectively analyzed 112 IM patients diagnosed at Fundación Valle del Lili in Cali, Colombia, the cases met the EULAR/ACR criteria. Data included demographics, clinical signs, laboratory findings, and malignancy. Malignancy associations were explored using logistic regression. The survival analysis was assessed using Kaplan–Meier curves and the Log-Rank test. Dermatomyositis was the most common subtype (45.5%), with a female predominance (66.1%). Cancer diagnosis occurred in 11.6% of cases, predominantly thyroid cancer. The median time from myopathy onset to cancer diagnosis was 11 months, with 75% of cases within the first year. Bivariate analysis indicated associations between cancer and age, Gottron's papules, digital ulcers, and heliotrope rash. However, multivariate analysis identified age as the only significant malignancy risk factor. Survival analysis showed better rates in younger patients. This study provides into the link between IM and cancer in the Colombian population. Thyroid cancer predominated, with a slightly higher proportion of female cancer diagnoses. Age emerged as a significant risk factor for malignancy. Understanding this association is crucial for early detection and improving patient outcomes related to IM-associated malignancies. [ABSTRACT FROM AUTHOR]

Objective: The increasing use of the internet has resulted in its gaining a more active role in both patient and professional education, including the YouTube platform, a popular platform for sharing and watching videos. This study aimed to evaluate the quality, usefulness, and reliability of videos on inflammatory muscle diseases on YouTube, as well as their determinants for both patient and healthcare professional education. Materials and Methods: The keywords "Inflammatory muscle disease", "Idiopathic inflammatory myositis", "Inflammatory muscle disease treatment", "Inflammatory myositis treatment", "Dermatomyositis" and "Polymyositis" were searched on the site on April 22nd, 2022. According to the Global Quality scale, three categories (high quality, middle quality, and low quality) were created based on the educational value of YouTube videos. The reliability of the information was assessed using the DISCERN tool. The video parameters were compared between the quality groups to reveal the determinants of the quality. Results: Seventy-five videos of patients and 116 videos of healthcare professionals were included in the video after exclusion. The numbers of high, intermediate and low-quality videos were 27 (36%), 27 (36%), and 21 (28%) for videos for patients, and 20 (17.2%), 60 (51.8%), and 36 (31%), respectively. Only the number of likes was found to be a determinant of video quality for healthcare professionals (p<0.05), and none of the other determinants were found to predict the quality for both groups (p>0.05). Conclusion: YouTube is an open platform for information, and it includes various educational videos of various quality. Experts, such as physicians, should be encouraged to provide more content to help both patients and healthcare professionals obtain better quality information. [ABSTRACT FROM AUTHOR]