The aim of this study was to determine the clinical, serological, and genetic features of anti-Jo-1 positive antisynthetase patients followed by a Hungarian single centre to identify prognostic markers, which can predict disease phenotypes and disease progression. It was a retrospective study using clinical database of 49 anti-Jo-1 positive patients. 100% of patients exhibited myositis, 73% interstitial lung disease, 88% arthritis, 65% Raynaud’s phenomenon, 43% fever, 33% mechanic’s hand, and 12% dysphagia. We could detect significant correlation between anti-Jo-1 titer and the CK and CRP levels at disease onset and during disease course. HLA DRB1⁎03 positivity was present in 68.96% of patients, where the CK level at diagnosis was significantly lower compared to the HLA DRB1⁎03 negative patients. HLA DQA1⁎0501-DQB1⁎0201 haplotype was found in 58.62% of patients, but no significant correlation was found regarding any clinical or laboratory features. Higher CRP, ESR level, RF positivity, and the presence of fever or vasculitic skin lesions at the time of diagnosis indicated a higher steroid demand and the administration of higher number of immunosuppressants during the follow-up within anti-Jo-1 positive patients. The organ involvement of the disease was not different in HLA-DRB1⁎0301 positive or negative patients who were positive to the anti-Jo-1 antibody; however, initial CK level was lower in HLA-DRB1⁎0301 positive patients. Distinct laboratory and clinical parameters at diagnosis could be considered as prognostic markers.

Overlap myositis is a distinct subgroup of idiopathic inflammatory myositis (IIM) with various clinical phenotypes. The aim of this study was to determine the clinical, serological, and genetic features of systemic sclerosis (SSc)‐IIM overlap patients. It was a retrospective study using clinical database of 39 patients, fulfilling both the criteria of SSc and IIM. 56.4% of the patients had limited cutaneous, 43.6% had diffuse cutaneous SSc, whereas 7.7% of the patients had dermatomyositis and 92.3% polymyositis. The two diseases occurred simultaneously in 58.97%, while 10.26% in myositis and 30.77% in scleroderma were initially diagnosed. The frequencies of organ involvement were interstitial lung disease 71.8%, dysphagia 66.7%, cardiac involvement 41%, pulmonary arterial hypertension (PAH) 30.8%, and renal involvement 12.8%, respectively. The presence of human leukocyte antigen (HLA) − DRB1∗03 and DQA1∗051∗01 alleles were significantly higher in the overlap patients than in healthy controls (82.35% vs. 27.54%; p < 0.0001 and 88.24% vs. 30.16; p < 0.0001). Certain clinical parameters, such as fever at diagnosis (41.67% vs. 7.41%, p = 0.0046), cardiac involvement (83.33% vs. 22.22%, p = 0.0008), subcutaneous calcinosis (41.66 vs. 11.11, p = 0.01146), and claw hand deformity (25% vs. 11.11%, p = 0.00016) were significantly associated with the presence of PAH. Upon comparison, the overlap patients and anti‐Jo‐1 positive antisynthetase patients showed similarities in terms of genetic results and major clinical features; however, SSc‐IIM overlap patients could be distinguished with higher erythrocyte sedimentation rate (ESR) level, more frequent presence of Raynaud’s phenomenon (p < 0.0001; OR: 20.00), dysphagia (p < 0.0001; OR: 15.63), and infrequent livedo reticularis (p < 0.01; OR: 0.11). SSc‐IIM overlap myositis is a unique group within IIM‐s possessing characteristic clinical features.

Background: Idiopathic inflammatory myopathies (IIMs), also known as myositis, are systemic autoimmune diseases characterized by chronic inflammation affecting the skin, muscles, and internal organs. Besides traditional risk factors and immune-mediated myocarditis, continuous activity of the immune system increases cardiovascular disease (CVD) risk, meaning that cardiovascular events are the leading causes of mortality in IIM patients. Statins are the most widely used lipid-lowering therapies, which reduce cardiovascular risk, but the fear of adverse muscular events inhibits the frequency of use. Methods: Our aim was to assess the CVD risk in a myositis cohort using the SCORE2 prediction system, carotid artery Doppler ultrasound measurement, and biomarkers; recommend individual lipid-lowering treatment; and follow the efficacy and adverse events of therapy in a 6-month treatment period. Results: The study population (80 IIM patients) was a middle-aged, female-dominant myositis cohort with an average disease duration of 9 years and low median global disease activity. Based on the SCORE2 evaluation, 78.8% of patients had medium/high CVD risk, while 73.13% had asymptomatic carotid plaque. After 6 months of adequate lipid-lowering therapy, 37.5% of patients reached a lower CVD risk category, the biomarker levels of atherosclerosis significantly decreased, and no progression in carotid plaques was detected. None of the patients reported an adverse muscular event or IIM relapse. Conclusions: Our findings proved that the CVD risk of patients with myositis is high, but carefully applied lipid-lowering treatment is the key to effective risk reduction. Risk stratification and the recommendation of preventive treatment are the responsibility of the treating physician.

Interferon signature is one of the key pathogenic pathways in idiopathic inflammatory myopathies (IIMs), particularly in dermatomyositis (DM). The aim of this study was to analyze Siglec-1, an interferon-inducible receptor, on different monocyte subsets in IIM subtypes and investigate its association with disease activity measures. Siglec-1 expression was measured by 8-color flow cytometry in 62 IIM patients and 10 healthy controls (HC). Disease activity was assessed using the International Myositis Assessment and Clinical Studies (IMACS) core set measures. Active DM patients exhibited significantly higher Siglec-1 mean fluorescence intensity (MFI) compared to inactive subgroups and HCs in every monocyte subset. Intermediate monocytes displayed the highest Siglec-1 expression across all groups and the strongest associations between disease activity markers. Siglec-1 expression on monocyte subsets was strongly associated with global, extramuscular global, constitutional, cutaneous, muscular, and gastrointestinal activity measures, but not with pulmonary, skeletal, and cardiac activities in the DM population. The best indicator of DM global disease activity among the examined biomarkers was Siglec-1 MFI on intermediate monocytes. Siglec-1 on intermediate monocytes correlates strongly with organ-specific clinical and biochemical markers of disease activity; therefore, it is a candidate biomarker for monitoring IIM disease activity. Siglec-1 could be useful in patient selection for interferon-targeted treatments.

Background: Idiopathic inflammatory myopathies (IIM) also known as myositis are systemic autoimmune diseases characterized by chronic inflammation affecting the skin, muscles and internal organs. Besides traditional risk factors and immune-mediated myocarditis, continuous activity of the immune system increases cardiovascular diseases (CVD) risk resulting that cardiovascular events are leading causes of mortality in IIM pa-tients. Statins are the most widely used lipid lowering therapies, which reduce cardio-vascular risk, but the fear of muscular adverse events inhibit the frequency of use. Methods: Our aim was to assess the CVD risk in a myositis cohort using the SCORE2 prediction system, carotid artery Doppler ultrasound measurement and biomarkers; recom-mend individual lipid-lowering treatment; follow the efficacy and adverse events of ther-apy in a 6 months’ treatment period. Results: Study population (80 IIM patients) was a middle aged, female dominant myositis cohort with an average disease duration of 9 years and low median global disease activity. Based on the SCORE2 evaluation, 78.8% of patients had medium/high CVD risk as well as 73.13% had asymptomatic carotid plaque. After 6 months of adequate lipid-lowering therapy 37.5% of patients reached lower CVD risk category, the biomarker levels of atherosclerosis significantly decreased and no progression in carotid plaques have been de-tected. None of the patients reported muscular adverse event or IIM relapse. Conclusion: Our findings proved that CVD risk of patients with myositis is high, but carefully applied lipid-lowering treatment is the key to effective risk reduction. Risk strati-fication and recommendation of preventive treatment is the responsibility of treating physician.

Background: Identifying novel, reliable blood-based biomarkers is essential to better assess disease activity in idiopathic inflammatory myopathies (IIMs). We aimed to determine serum fatty acid binding protein 3 (FABP3) levels in IIM patients and evaluated their correlations with disease activity, muscle strength, and conventional biomarkers. Methods: This cross-sectional study enrolled 80 patients with IIM (49% polymyositis, 51% dermatomyositis) and 47 gender-matched healthy controls. Disease activity was evaluated using the IMACS core set measures, and serum FABP3 levels were determined via ELISA. Results: FABP3 levels significantly correlated with creatine kinase (CK; r = 0.42, p < 0.001), high-sensitive C-reactive protein (hsCRP; r = 0.35, p = 0.002), and physician global disease activity (PhG; r = 0.28, p = 0.013), while showing a strong negative correlation with manual muscle testing (MMT; r = −0.39, p = 0.0005). Conversely, CK and hsCRP failed to correlate with disease activity or muscle strength. Statistical analysis confirmed that FABP3 is an independent predictor of muscle strength, and correlates with PhG better than CK in patients with chronic myositis. Conclusions: FABP3 is a promising biomarker for IIM disease activity, which may enable more effective therapeutic decision-making in clinical practice.