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Heart Disease in Myositis: Causes, Symptoms & Treatments

Explore the link between myositis and heart disease, including prevalence, causes, and diagnostic techniques, such as MRI and ECHO. Learn about the symptoms of coronary heart disease and subclinical heart dysfunction in myositis patients.

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Heart Disease in Myositis: Causes, Symptoms & Treatments

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  1. Myositis and heart disease

  2. Louise Pyndt Diederichsen, MD, PhD Center for Rheumatology and Spine Diseases Copenhagen University Hospital Rigshospitalet Copenhagen, Denmark

  3. Size • Denmark: 42.933 km² • Minnesota: 225.181 km² • USA: 9.834.000 km²

  4. Myositis – a systemicdisease

  5. Background – what do we know? • Increasedmortality in myositiscompared to the general population • Cardiovascular diseases one of the dominant causes of death • Prevalence and type of heart involvement in myositis • Cause and treatment ?

  6. Background – disease mechanisms • Atherosclerosis • Inflammation of the heart muscle

  7. Atherosclerosis - imaging Vessel - artery Plaque Atherosclerosis Heart arteries CT scanner Heart artery calcification (HAC)

  8. Symptoms of coronary heart disease -CHD • The main symptoms of CHD are: • chest pain (angina) • heart attacks • heart failure • You can also experience other symptoms, such as heart palpitations and unusual breathlessness • But not everyone has the same symptoms and some people may not have any before CHD is diagnosed American Heart Association, https://www.heart.org

  9. Heart artery calcification (HAC) in myositis • Cross-sectional study of 76 patients with poly or dermato-myositis and 48 healthy controls measuring heart artery calcification (HAC) by CT-scan Diederichsen et al. Arthritis Care Res. 2015

  10. Heart artery calcification (HAC) in myositis • High HAC score more frequent in patients with PM/DM (20%) than in healthy controls (4%) Sectional CT images of the upper body

  11. Heart artery calcification (HAC) in myositis • 33% of the myositis patients were obese compared to 11% of the controls • Hypertension and diabetes were more frequent in patients vs. controls (71 % vs. 42% and 13% vs. 0%) • High HAC score not associated to the myosit disease per se, but to traditional cardiovascular risk factors as higher age and smoking

  12. Treatment - and Your Risks to Prevent a Heart Disease American Heart Association, https://www.heart.org • Major risk factors that can’t be changed: • Increasing Age • Male gender • Heredity • Major risk factors you can modify, treat or control: • Tobacco smoke  • Physicalinactivity • High bloodpressure • Obesity and beingoverweight • Diabetes  • (High bloodcholesterol)  • https://www.heart.org/en/health-topics/heart-attack/understand-your-risks-to-prevent-a-heart-attack +

  13. Background – disease mechanisms • Inflammation of the heart muscle  myocarditis Heart muscle

  14. Myocarditis – diagnostic methods • Biopsy of the heart • Heart Magnetic Resonance Imaging (MRI) www.hopkinsmedicine.org

  15. Myocarditis – Magnetic Resonance Imaging (MRI) • Heart biopsy • Heart MRI MRI scanner

  16. Heart function

  17. Heart function • Function of the heart separated in two phases: • Diastolic function (filling phase), relaxed • Systolic function (pumping phase), contracted

  18. Heart function - Echocardiography • Heart function evaluated by ECHO: ultrasound measure of the heart • Systolic heart function (ejection fraction (EF) • Diastolic function

  19. Systolic heart function - ECHO • Systolic heart failure due to myositis is well-known, but rare • The most common symptoms of heart failure are: • breathlessness – this may occur after activity or at rest; it may be worse when you're lying down, and you may wake up at night needing to catch your breath • fatigue – you may feel tired most of the time and find exercise exhausting • swollen ankles and legs – this is caused by a build-up of fluid (oedema); it may be better in the morning and get worse later in the day • Subclinical (without symptoms) systolic heart dysfunction more common American Heart Association, https://www.heart.org

  20. Subclinical systolic heart dysfunction • 30 patients with newly diagnosed polymyositis and dermatomyositis • ECHO at baseline and after 3 months of immunosuppressive treatment (Prednisolone (100%), Cyclosporine 13%) • At baseline, subclinical systolic dysfunction in patients - compared to healthy controls – which normalized after 3 months of immunosuppressive treatment (Péter et al. J Reumatol. 2015)

  21. Subclinical diastolic heart dysfunction • Decreased diastolic heart function in patients with myosit compared to healthy controls, detected by ECHO • Decreased diastolic heart function associated to presence of myositis autoantibodies • Increased heart rhythm and conduction disturbances in patients with myosit compared to healthy controls, detected by ECG • Heart findings might be induced by inflammation replaced by fibrosis, clinical significance unknown Diederichsen et al. Arthritis Care Res. 2016

  22. Recommendations – screening & treatment • Atherosclerosis: increased attention payed to traditional cardiovascular risk factors, following general guidelines • Stop smoking • Exercise • Medical treatment: • Diabetes • Hypertension • Inflammation of the heart muscle: • At diagnosis, screening for heart involvement • ECG (electrocardiogram) • blood samples including heart markers • ECHO (ultrasound of the heart) • During disease course, if cardiac symptoms + + If abnormalheart MRI (biopsy)

  23. Treatment of myocarditiscaused by myositis • The rheumatologist/neurologist: • Conventional immunosuppressive treatment of myositis • Prednisolone • Disease-modifying anti-rheumatic drugs (DMARDs): methotrexate, Azathioprine, cyclo-phosphamide, Mycophenolatmofetil, Rituximab, cyclosporine • The cardiologist: • Conventional medical treatment of • heart failure and/or • conduction and rythm abnormalities Global Myocardial Edema in Antisynthetase Syndrome detected and monitored by heart MRI Sado DM et al, Circulation, 2016

  24. Perspectives • Longitudinal studies of heart affection in larger myositis patient cohorts are needed, in an international set-up (https://euromyositis.eu/) • Prevalence and type of clinical and subclinical heart affection • Cardiac biomarker(s) – autoantibodies? • Treatment of heart involvement • Algorithm of cardiac screening

  25. PROJECT MYOSCLHeart Involvement andAutoantibody Profile in Myositis Sine Søndergaard Korsholm MD, futurePhD student Center for Rheumatology and Spine Diseases Copenhagen University Hospital Copenhagen, Denmark

  26. Primaryaim To identify biomarkers for heart involvement in myositis

  27. Heart measures Rhythm and conduction disturbances by ECG: Blood samples: • Autoantibodies • Cardiacdamage markers: Troponins Heart structure and function by heart MRI:

  28. Hypotheses • Specific myositis-autoantibodies correlate with the presence of heart abnormalities detected by: • Electrocardiogram - ECG • Blood samples with heart damage markers - troponins • Heart MRI • Heart abnormalities detected by heart MRI are sensitive to immunosuppressive treatment

  29. Preliminary results (DK, SE) Risk factors for ECG abnormalities in myositis and systemic sclerosis

  30. Perspectives • Identification of a heart biomarker gives the opportunity for:

  31. Thanks to • Personnel and patients at • Karolinska University Hospital • Ingrid Lundberg • Maryam Dastmalchi • Louise Ekholm • Daniel C. Andersson • Martin Ugander • Magnus Lundin • Salford Royal NHS Foundation Trust • Hector Chinoy • James Lilleker • Rigshospitalet • Sine Søndergaard Korsholm • Søren Jacobsen • Nanna Witting • Rasmus Møgelvang • Katrine Aagaard Myhr • Markus E. Krogager • Jesper Helbo Storgaard • Odense University Hospital • Redi Pecini • Axel C. P. Diederichsen • John Bonde Knudsen • Eva Simonsen • Jane Simonsen • Svend Hvidsten • Oslo University Hospital • Helena Andersson • ØjvindMolberg • OtherCollaborators • Statens Serum Institut • Tina Friis • EuroMyositis • Niels Steen Krogh • Funds • The Danish Rheumatism Association • King Christian X’s Fund • Lysgaard Foundation • Research Funds of Committee of Chief Physicians, Odense University Hospital (OUH) • OUH Internationalisation Fund • OUH Pregraduate Fund

  32. Thankyou for your attention! Louise Pyndt Diederichsen, MD, PhD Center for Rheumatology and Spine Diseases Copenhagen University Hospital Rigshospitalet Copenhagen, Denmark

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