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.

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.

Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we present a case of 50-year-old female patient with long-term asthma treatment who presented initially with extreme eosinophilia (56%) and severe progressive ascending paresis, similar to Guillain–Barré syndrome, leading to tetraplegia. After navigating through diagnostic mazes, the diagnosis of EGPA was established based on eosinophilia, myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) positivity, asthma, eosinophil granulomatosis in the gastrointestinal tract, and severe peripheral nervous system involvement, complicated with rare central nervous granulomas and ischemia. With combined immunosuppressive and immunomodulatory treatment including high-dose corticosteroids, rituximab and intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation over 6 months, our patient recovered gradually from tetraplegia and adverse events such as severe infections and osteoporotic fractures. Now, from a 2-year perspective, we can conclude a successful treatment leading to decrease in all of her symptoms. Due to persistent eosinophilia after steroid tapering, she was switched to mepolizumab maintenance treatment and demonstrated continuous improvement of motor and sensory functions. Thanks to periodically repeated rehabilitation, she became self-sufficient and returned to her previous job. Our case highlights that EGPA patients should be treated in a center of expertise due to the rarity of the disease and complexity of diagnosis and treatment. Careful multidisciplinary cooperation, the huge effort of the patient, and a supportive environment can show a way back from immune-mediated tetraplegia.

Central nervous system (CNS) involvement is an extremely rare manifestation in eosinophilic granulomatosis with polyangiitis (EGPA), associated with a poor prognosis. Here we present a case of 50-year-old female patient previously long-lasting treated with asthma who presented initially with extreme eosinophilia (56%) and severe progressive ascending paresis similar to Guillain-Barré syndrome leading to tetraplegia. After navigating through diagnostic mazes; the diagnosis of EGPA was established based on eosinophilia, anti-neutrophil cytoplasmic antibody positivity (MPO-ANCA), asthma, eosinophil granulomatosis in the gastrointestinal tract, and severe peripheral nervous system involvement complicated with rare central nervous granulomatosis and ischaemia. With combined immunosuppressive and immunomodulatory treatment including high-dose corticosteroids, rituximab and intravenous immunoglobulin along with symptomatic treatment and planned rehabilitation over 6 months our patient recovered gradually from tetraplegia and adverse events such as severe infections and osteoporotic fractures. Now from a 2-year perspective we can conclude a successful treatment leading to decrease in all of her symptoms. Due to persistent eosinophilia after steroid tapering, she is currently on mepolizumab maintaining treatment and demonstating continuous improvement of motor and sensory functions. Thanks to periodically repeated rehabilitation, she became self-sufficient and returned to her previous job. Our case represents that EGPA patients should be treated in a centre of expertise due to the rarity of disease and complexity of diagnosis and treatment. Careful multidisciplinary cooperation, huge effort of the patient and supportive environment can show a way back from immune-mediated tetraplegia.