We are pleased to introduce Dr. Christina Charles-Schoeman today to answer your questions about Issues of the heart: A discussion of heart disease and myositis. Dr. Charles-Schoeman, a member of TMA's Medical Advisory Board, is a rheumatologist and associate professor of medicine at Ronald Reagan UCLA Medical Center in Los Angeles. She is an active investigator in multiple clinical trials involving novel therapies for myositis, and her research in this area includes the study of cardiovascular disease in myositis, including the use of cholesterol-lowering statin drugs and their association with myopathy. Thank you for joining us today, Dr. Charles-Schoeman.Ask a Question
Dermatomyositis and Sjögren's SyndromeParticipant:
I have dermatomyositis and Sjögren's syndrome, along with other autoimmune diseases. When I have a flare, I struggle with my breathing and, when standing, my heart races and I have severe fatigue in my chest and other muscles to the point that I immediately have to lay down. To get relief from this feeling, laying down is the only relief. Sitting doesn't help me; I have to put my recliner all the way back and lie flat. When I am not flaring, I still feel this way when I have done too much activity. I have discussed this with some of my doctors, and I never really get an answer as to what this is from. My heart tests have always been pretty normal. I go this week (February 1st) for another echo Doppler ordered by my rheumatologist to be sure nothing more is happening. My question is, what is your opinion as to what is causing this issue, and do you see it in other people who suffer with these diseases? Thank you.Dr. Christina Charles-Schoeman:
I am not sure without seeing you and doing a full history and physical examination. However, I have some questions for you to discuss with your rheumatologist: Have you had a stress test of the heart? Are your pulmonary tests and chest CT OK? Do you have any trouble with weakness of your diaphragm muscles? Is it possible you are deconditioned, and is it safe to start a monitored, gentle, cardio-pulmonary rehabilitation program?
Do you know of any data (or have an opinion) on the use of the new drug Entresto for treating advanced heart failure (EF of 15 - 20%) caused by polymyositis? In my case the polymyositis is in remission, but the damage to the heart has been done.Dr. Christina Charles-Schoeman:
Entresto is a combination of sacubitril and valsartan. Sacubitril is a blood pressure medicine, and valsartan is an angiotensin II receptor blocker (sometimes called an ARB). Valsartan keeps blood vessels from narrowing, which lowers blood pressure and improves blood flow. I do not know of any specific studies looking at heart failure medications in myositis patients, however, I would follow your cardiologist’s recommendations in treating the heart failure itself.
Clogged Artery PreventionParticipant:
I have recently gone back on statins after being taken off the drug when diagnosed with DM in 2007. What should I be looking for to ensure no adverse myositis-related reactions? Are there alternatives to statins for elevated cholesterol that prevent plaque build-up in the arteries? I was put back on statins as Zetia did not prevent me having a clogged carotid artery last year.Dr. Christina Charles-Schoeman:
See my previous response for other lipid-lowering agents. When initially starting a statin in a myositis patient, I always monitor the CPK closely as well as the patient’s clinical symptoms to make sure the myositis is not worsening. We published a study previously showing that nearly 80% of myositis experts (belonging to the International Myositis Assessment and Clinical Studies Group (IMACS) group) use statins in their myositis patients.
IBM and Heart Muscle DiseaseParticipant:
Please discuss IBM and heart muscle disease.Dr. Christina Charles-Schoeman:
There is not a lot of published data specific to IBM alone, however, data on IIM as a group of diseases suggests that myositis can affect the heart muscle in some cases. There is one Norwegian study that suggested that mortality due to heart failure and heart attacks is higher in IBM patients compared to DM and PM patients. However, the age of IBM patients was higher in that study; this can confound the data. In addition, the study size was small overall, as is true of most myositis studies.
I was diagnosed with DM in March 2016 and have been having more frequent episodes and increase in severity of left-sided chest pain and shortness of breath (nonsmoker, not overweight, former runner). I had a nuclear stress test last month that showed basal septal ischemia. Coronary artery disease (CAD) was ruled out on subsequent cardiac catheterization. What is the likelihood that this is vasospastic angina associated with my DM? And if so, what kind of questions should I be asking my new cardiologist at my appointment tomorrow?Dr. Christina Charles-Schoeman:
I found one previous case report of a woman with dermatomyositis who also had vasospastic angina (see link below). Certainly because DM involves the vasculature, a possible link should be considered and your DM-associated inflammation should be aggressively treated. Our research is very interested in understanding the vasculature better in DM patients. As far as treatment goes, calcium channel blockers are the first line therapy for vasospastic angina. Nitrates are also useful in alleviating symptoms. https://www.ncbi.nlm.nih.gov/pubmed/?term=vasospastic+angina+and+myositis
Cardiac Disease PredictionParticipant:
1) Since your research has looked at the abnormality of HDL in patients with dermatomyositis, if our HDL number is in the normal range, is there some other easily accessible marker to predict problems with potential cardiac disease, such as c-reactive protein test (CRP)? 2) Do any of the treatments used for dermatomyositis have a positive impact on the cardiac risk? Thank you.Dr. Christina Charles-Schoeman:
This is a complicated topic but one our lab is addressing. CRP has been used as a measure of cardiovascular risk in the general population. There is no data yet that I know of specifically about CRP and myositis. We are working on HDL “function” assays that look at how well the HDL particles prevent atherosclerosis—not just the number of the HDL particles or the amount of cholesterol, which may be normal on routine testing. We recently showed (published research in Arthritis and Rheumatology 2017) that improvement in disease activity in patients with rheumatoid arthritis with treatment was associated with improvement in HDL function. We hope to publish something soon in myositis patients and are looking at further work on the effects of myositis treatment. Hopefully, more will follow.
Thank you for your time. I would like to know if arrhythmias are common among myositis patients. I have multifocal ventricular tachycardia. Can this affect my heart function? Am I at higher risk for heart failure (since the heart is also a muscle)? Are there any cardiac medications specifically antiarrhythmics that should be avoided in myositis patients?Dr. Christina Charles-Schoeman:
Inflammation can occur in the heart muscle in myositis patients. This can lead to fibrosis of the heart (damage), which can lead to arrhythmias (like MVT and others), and poor heart function (also called heart failure). Uncontrolled arrhythmias are not good for the heart. Most important is to control myositis as best as possible and closely follow with a cardiologist. I do not know of specific antiarrhythmics that should be avoided in myositis patients; I would check with your cardiologist.
Slow Progressing sIBMParticipant:
Hello. I have slow progressing sIBM (age 70, diagnosed in 2008), and my doctor has just taken me off a low dose (5mgs) statin and replaced with what he calls a new, more effective, less risky medication called ezetimibe 10 mgs. Can you comment please? Why don't I know of or see others using this medication? I live in Queensland, Australia. I have no currently diagnosed heart problems.Dr. Christina Charles-Schoeman:
See my previous answer about the other cholesterol agents. Ezetimibe (“zetia”) is a cholesterol absorption inhibitor that impairs cholesterol absorption in the intestine that has been studied mostly as additive therapy to statin therapy. I do not think that there is any data suggesting that it is “more effective” than statins. Many doctors are fearful about using statins in patients with myositis. I would ask your doctor if there is a specific reason why he/she made the change.
I have extremely high cholesterol that does not lower with diet/exercise. I am on Lipitor 80 mg. My cardiologist told me I need it. Is there any other treatment?Dr. Christina Charles-Schoeman:
Yes, however, most doctors consider statins (the type of drug you are taking) to be “first line” therapies because of the great benefits (decreases in heart attacks, cardiovascular death) they’ve shown in clinical trials. Other treatments include bile acid sequestrants, (used for mild-moderate elevations in the “bad” cholesterol; side effects include GI issues, such as nausea, bloating, cramping; also increased liver tests), niacin (often poorly tolerated due to flushing in 80% of patients, itching, etc.), ezetimibe (“zetia”) a cholesterol absorption inhibitor that impairs cholesterol absorption in the intestine (has been studied mostly as additive therapy to statins), and PCSK9 inhibitors (monoclonal antibodies that inhibit a protein called PSCK9 and thereby lower LDL “bad” cholesterol levels; injectable medications that have been recently approved for specific patients groups).
Cardiac Arrhythmias with sIBMParticipant:
Cardiac arrhythmias with sIBM. When I have been stressed at work on a couple of occasions I have felt dizzy and my heart rate was in the 40's on the heart monitor (sinus rhythm).Dr. Christina Charles-Schoeman:
Involvement of the heart muscle causing arrhythmias occurs in idiopathic inflammatory myopathies (IIM) [including IBM] and therefore you should be evaluated/followed closely by a cardiologist. Some patients do need a pacemaker when their heart rate goes that low and they are symptomatic.
Since being diagnosed with polymyositis, I have been taken off of Crestor, and my lipid profile numbers have greatly increased. I have tried non-statin cholesterol drugs, but they have not been very effective, and besides that, they are very expensive and typically not covered by my insurance. I have tried taking 5mg Crestor 3 days a week, but my CK has increased. Do you have any suggestions?Dr. Christina Charles-Schoeman:
Have you been tested for the anti-HMGCR antibody? If negative, you could consider a trial of another “less effective” but sometimes better tolerated statin such as pravastatin. Start low and gradually increase dose. Also, I would follow closely the CK, but be sure to realize there is some normal variation from blood draw to blood draw.
Complete Heart BlockParticipant:
Is there any known association between polymyositis and complete heart block? Although I realize there are many causative factors to heart block, I'm curious about any relationship. I was diagnosed with PM in 2012, am currently on azathioprine and prednisone, consider my PM stable, but had a sudden onset of complete heart block with a syncopal episode in December and ended up with a dual-chamber pacemaker. Just curious! Thank you.Dr. Christina Charles-Schoeman:
Conduction abnormalities on ECG are the most commonly reported evidence of heart problems in IIM. So, yes, the polymyositis could have caused inflammation in the heart and lead to some damage that could have caused the heart block. This can occur when the disease is active as well as in remission.
Sharp Heart PainParticipant:
I have DM (mda-5 antibody) with interstitial lung disease (DLCO at 72% and VC at 106%). When I inhale deeply I sometimes feel a sharp pain on the lower right hand side of the heart (or the left breast), and the pain shoots up into the left side of my neck. Is this linked to a heart condition or just part of the muscular symptoms of DM? My last visit to the cardiologist was normal, but he is not a myositis specialist.Dr. Christina Charles-Schoeman:
It’s hard to say exactly what this is without further discussion and examining you. It could be musculoskeletal, involving the muscles or the sternal “rib” joints, or it could be lung-related due to inflammation, etc. I would make sure your rheumatologist focuses on it and does the appropriate exam/work-up at your next appointment.
Thickened Heart Muscle Walls with PrednisoneParticipant:
After taking prednisone for about 13 years I was told I have thickened heart muscle walls. Is this a side effect of the drug or common with myositis?Dr. Christina Charles-Schoeman:
Prednisone is used to reduce the inflammation in the muscles, including the heart muscle. In my opinion, it is less likely to cause thickening of the heart muscle, although I do not know that this has been specifically studied. Stiffening of the heart muscle walls can occur from the myositis itself. Also, other common things like hypertension can lead to “thickened heart muscle walls.”
HDL Cholesterol LevelsParticipant:
Would the addition of fish oil in the diet be helpful in this situation regarding the abnormal HDL?Dr. Christina Charles-Schoeman:
Fish oil has been shown to increase HDL cholesterol levels. I do not know of any data on HDL function. Several years ago we published data showing that atorvastatin (Lipitor) improved HDL function in RA patients.
I know statin drugs can cause a kind of myositis called necrotizing myopathy. Does this statin-induced myositis go away if you stop taking stains? If not, does it ever improve or go into complete remission? Does it get worse the longer you take statins? And what is one to do if you have to take statins for cholesterol problems?Dr. Christina Charles-Schoeman:
The initial description of necrotizing myopathy with statin drugs was based on patients whose myositis responded to therapy with immunosuppressive drugs and most of them had had statin exposure. The antibody was identified from these patients. Patients with “statin” myositis should never take statins, and they need immunomodulatory treatment for their myositis. I treat several patients with this disease, and they have improved greatly with treatment. Luckily, this is a VERY rare disease and does not occur for the vast majority of patients taking statins.
Prednisone and CADParticipant:
Does prednisone contribute to CAD?Dr. Christina Charles-Schoeman:
Yes, HOWEVER prednisone is very important in controlling the disease itself, which also contributes to heart disease. For this reason other immunosuppressant agents are also used in myositis—cellcept, methotrexate, IVIg, etc.—to limit the prednisone dosage.
Does myositis affect cardiac muscle the same as skeletal muscle, and why does sIBM seem to mostly affect the quadriceps?Dr. Christina Charles-Schoeman:
Great question. The answer is unclear at present as we do not have enough data comparing the heart and skeletal muscle in IBM. There is more work to be done.
Myositis and Heart DiseaseParticipant:
Is there a correlation between those who have myositis and heart disease?Dr. Christina Charles-Schoeman:
Yes, as is true of other autoimmune inflammatory diseases, coronary artery disease (atherosclerosis) appears to be more prevalent in patients with myositis. The myositis itself can also affect the heart. See previous responses.
Risk of Cardiovascular DiseaseParticipant:
Does having DM and using prednisone and methotrexate increase my risk of cardiovascular disease? I was low risk before the DM diagnosis in December 2016, do not take statins and am normal weight, exercise, etc. I am a 70 y/o female.Dr. Christina Charles-Schoeman:
See previous responses. Interestingly, there is a large NIH-sponsored clinical trial ongoing to see if methotrexate may actually be protective for heart disease.
For a person who already has cardiovascular disease (CVD), is the concern for PM developing in the area of the chest wall of greater concern? Is it a natural progression? Background: I am 52 and recently began having chest pain that responds to nitrostat, but my cardio team says my heart is in good shape (I just had stress test). I have familial hypercholesterolemia, cannot take statins, rely on LDL apheresis, and I had a four-vessel coronary artery bypass graft three-and-a-half years ago. I am concerned that my cardiologists and neurologists are not on the same page, and I don't know what to do or if I should do anything differently.Dr. Christina Charles-Schoeman:
I do not know of any data that CVD would predispose to PM developing in the chest wall muscles versus other muscles.
I have PM (12 years) and systemic lupus erythematosus (SLE) and have just been diagnosed with AFib. Would PM possibly have a role in this development?Dr. Christina Charles-Schoeman:
It is possible that the PM could have damaged the heart and predisposed to AFib. It is unclear, however…most patients develop AFib without PM.
Risk of Heart DiseaseParticipant:
How can I lessen my chance of heart disease with dermatomyositis.Dr. Christina Charles-Schoeman:
Work with your rheumatologist to control the myositis as best as possible and make sure to work on “traditional” cardiovascular risk factors: monitor blood pressure, maintain healthy weight, don’t smoking, exercise, etc.
I was diagnosed with antisynthetase syndrome in March 2011. I had an echocardiogram two weeks ago and will meet with a cardiologist next week. My pulmonologist said that there is no evidence of pulmonary hypertension, but I have a hole in my heart. I believe the diagnosis is "LVEF-TTE TRANSTHORACIC ECHO 75." What questions should I ask the cardiologist? What type of information should I receive?Dr. Christina Charles-Schoeman:
I would ask for the specific diagnosis (what you copied looks like the type of test) and what needs to be done for treatment and future monitoring and why.
Recurrence of AFibParticipant:
I was diagnosed with PM nine years ago; after a muscle biopsy it was changed to DM without skin involvement. I am Jo-1 positive. Two years ago I was diagnosed with atrial fibrillation (AFib) and put on medication. Last May, I had an ablation and have had no AFib symptoms since then (checked with multiple EKGs including a month-long EKG). Since I have myositis, am I at a higher risk for a recurrence of AFib than one would be without an autoimmune disease?Dr. Christina Charles-Schoeman:
It’s hard to say! You may be if you have a lot of damage from the myositis. However, I would not fret over this but rather just follow closely with your cardiologist as well as your rheumatologist for good monitoring and disease control.
IBM Affecting the HeartParticipant:
I was diagnosed with inclusion body myositis a few years ago. I still walk unaided, but I fall over sometimes. Normally I know why I have fallen. Two weeks ago I fell rather heavily. The ambulance people said I had very low blood pressure immediately afterwards (90/60), and that there was something with my ECG that they wanted to get checked in hospital. Whilst in hospital, my ECGs became clear, but blood tests showed "Troponin T was elevated – 136, and CK was elevated – 347." This does not mean anything to me but has led to discussion between doctors as to whether I may have had a "silent heart attack" or whether it is related to my IBM. One doctor found research that suggests that, in rare cases, IBM can affect the heart. Is this the case? Can you tell me if the indicators described above are likely to be related to IBM? If so, what should I expect in the future regarding collapses? If it's not related to IBM, could the IBM account for the blood test results shown above? I would really appreciate any information you can give me. By way of context, I have since had a number of tests and will be having an appointment with a cardiologist to discuss the results on Friday, 10 February.Dr. Christina Charles-Schoeman:
The fact that you don’t remember the fall (in contrast to others) plus the low BP is concerning for an arrhythmia or other “heart” issue. Please see my previous comments regarding IBM and heart disease. Additional thoughts: Troponin T is a lab test that is not specific to the heart muscle; it can also be expressed in skeletal muscle (like CK). In people without myositis, an elevated Trop T may suggest a heart attack. However, in you it could be related to the ongoing inflammation/damage in skeletal muscle (since your CK was also high). Hence, Trop I is a better test; it appears to be more specific to the heart (not elevated by your myositis). I would ask if this test could be done on any remaining stored serum (blood). Because heart disease (including arrhythmias, atherosclerosis, etc) is a significant concern in myositis patients (as discussed above). I would also ask your doctors for a complete cardiac work-up: stress test with echo, cardiac (“Holter”) monitor to assess arrhythmias, etc. Hopefully you have already done these.
Atrial Septal Defect ComplicationsParticipant:
I have PM. I also have severe obstructive sleep apnea, pulmonary hypertension (PH), an atrial septal defect (ASD), diastolic dysfunction, AFib/flutter, right heart failure, right bundle branch block (RBBB), and a pacemaker. I am concerned that my ASD is contributing more to my pulmonary and heart issues than is known. I do not know how large the ASD is, but I do know that it is a left-to-right shunt. After researching congenital heart defects, it appears that this can cause pulmonary hypertension and AFib/flutter, with the symptoms becoming noticeable in the fourth decade. What is the possibility of this being an issue, and how do I find out more info? My doctor now wants to treat the PH and do an ablation (which I am not a big fan of).Dr. Christina Charles-Schoeman:
You have a complicated situation due to the ASD. I find it always very useful for patients to get a second opinion from an established expert in the area, if they can do so. For you, this would be a cardiologist very familiar with ASD cases. This may help you proceed with any treatment recommended.
My heart became quickly and rapidly deconditioned during the acute onset of the DM. I am increasing my exercise tolerance, now 11 months out. I did NOT develop an arrhythmia. I have noticed that if I am not very persistent with aerobic exercise, I seem to loose what aerobic capacity I have gained very quickly. Is the heart muscle just like my skeletal muscles in that regard? What is the optimal amount of time to exercise aerobically so as to strengthen the heart muscle but not break down skeletal muscle?Dr. Christina Charles-Schoeman:
Both the heart and skeletal muscle need exercise! There is not a specific amount of time for this; it is different for each person. I find it very useful for my patients to start in a monitored cardio-pulmonary rehab program and then continue on their own.
What is you view on whether Praluent, the new once-a-month injection for hyperlipidemia, can be safely used in patients with sIBM who took statins long-term prior to diagnosis? Does the fact that Praluent works differently than statins make a difference? Do you prescribe Praluent to your patients with sIBM? If so, does the presence or absence of statin antibodies in the patient play a part in your decision to prescribe or not prescribe Praluent to them?Dr. Christina Charles-Schoeman:
There is no data on this to date, HOWEVER, I think Praluent is a reasonable alternative to statins as it has a completely different mechanism of action. One of my patients intolerant to statins is currently working with cardiology to get it approved.
SIBM and the HeartParticipant:
Can sIBM affect the heart? Also, would you know if the progression of sIBM can be slowed down or go into a remission phase?Dr. Christina Charles-Schoeman:
Yes, IBM can affect the heart. Please see my other responses. It is best to be monitored closely with a baseline echocardiogram, etc. It’s hard to predict the course.
Dizzy, Breathless and Struggling to WalkParticipant:
My consultant has taken me off prednisolone completely and is now weaning me off azathioprine. (I have dermatomyositis.) I have dizzy spells, am breathless, and struggling to walk, among other symptoms. Do you think these symptoms are related to a heart issue, or are they direct dermatomyositis symptoms?Dr. Christina Charles-Schoeman:
It is very important since you are being weaned off medicines that you be evaluated to see if the DM is more active. Are you having typical symptoms again? Have your lungs been evaluated? It’s also important that you have a COMPLETE cardiac work-up: stress test, etc.
Thank you Dr. Christina Charles-Schoeman for taking time out of your busy schedule to answer questions for TMA members. This concludes today's discussion. Thanks to all of the members who participated.Dr. Christina Charles-Schoeman:
Thank you for the terrific session. My very best wishes to all of you!!! Christina