Issues of the Heart: A Discussion of Heart Disease and Myositis

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.

I do not know of any data that CVD would predispose to PM developing in the chest wall muscles versus other muscles.

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.

See previous responses. Interestingly, there is a large NIH-sponsored clinical trial ongoing to see if methotrexate may actually be protective for heart disease.

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.

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.

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.

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 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.

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.

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.

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.

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.

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.

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.

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.

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 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

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.

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.

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.

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?

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.

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.

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.

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.

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.”

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.

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.

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).

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.