Before the March meeting of the Global Conference on Myositis 2019 in Berlin, Germany, TMA asked its members to submit questions they would like to pose to the researchers and clinicians gathered at this meeting. You sent us nearly 80 questions, and we submitted them to the program organizers, seeking answers. A number of myositis experts responded. The following are their answers. Please note that experts do not always agree, so some responses offer differing opinions.

Correlation between myositis and other diseases/symptoms:

  • Is there any correlation between myositis and sleep apnea?

Yes, it is possible that there may be an association between myositis and sleep apnea, but it is rare.

  • Is there any connection between autoimmune hemolytic anemia (AIHA) and polymyositis?

A few rare cases of patients who have both of these conditions have been reported, but it is very rare. Neither of these conditions has been found to cause tooth decay. We have no data on life expectancy these patients, but if the diseases are treated well, we would expect no reduction in life expectancy.

  • I was just diagnosed with lupus after 2012 confirmation of sIBM. Is it typical for autoimmune diseases to occur after diagnosis of IBM?

People sometimes develop more than one autoimmune disease. This seems unusual, however. Both diseases should be reconfirmed to show/exclude overlaps.

  • I have an HIV-positive diagnosis and IBM. Although I’m extremely healthy with T cell counts higher than those without the disease, I still feel as if somehow the HIV caused my IBM. Is there any research around IBM as it relates to HIV, and could the HIV meds be causing the muscle weakness?

There are only few case reports showing this combination, so it is unlikely, but possible that there may be a causal relationship.

Some (older) meds can cause mitochondrial damage in HIV, and sIBM is a rather rare but well-established coincidence. We think viral diseases, such as HIV and hepatitis B and C, can cause sIBM.

  • Is restrictive lung disease seen as part of polymyositis or another type of myositis?

Different subtypes of interstitial lung diseases (ILD) can be associated with antisynthetase syndrome, and this causes lung restriction. ILD can occur with several forms of myositis as well as other autoimmune diseases.

  • Please comment on the link between IBM and large granular lymphocyte (LGL) leukemia. I have both and have read that there are others like me.

This is a very rare combination. Both are linked to pathogenic cytotoxic CD8+ T cells.

LGL lymphocytes are associated with sIBM in variable quantities (sometimes many, sometimes very few or none, BUT this does not mean that all sIBM patients have LGL leukemia. Conversely, not all LGL leukemia patients will get sIBM, but some (very few!) have been described and at younger age than ‘normally’ possible.

  • Have any of the experts heard of a patient with dermatomyositis (with ILD) who is also being treated for a thoracic aortic aneurysm and postural orthostatic tachycardia syndrome (POTS)? Do they have any thoughts, or is there any published literature or studies on whether these conditions are somehow related, and/or treatment options?

This combination of conditions is by chance.

  • I am 26 years old and diagnosed one year ago with polymyositis in addition to interstitial lung disease (ILD). I am from the United Arab Emirates. Due to lack of knowledge regarding such disease around here, I’m the only one around who has it.

This is probably antisynthetase syndrome.

1. What do I need to expect in the future?

You should have an intensive myositis workup and adequate treatment with medications as soon as possible.

2. Will I keep feeling this continuous pain?

With therapy, symptoms should become better.

3. Will my joints be affected?

Joint involvement could happen if you develop an overlapping condition such as rheumatoid arthritis.

4. Will I have the chance to live like any other lady without difficulties, like having kids for instance? 

Yes, you should be able to live a normal life and have children, but first your disease must be controlled.

  • My doctor says he is 80% sure I have IBM. I was simultaneously diagnosed with breast cancer, which has since been treated. Is there any treatment, experimental or not, that I can try for IBM?

There are clinical trials currently underway, but most likely tumors will exclude you from participating.

1. Will any of these treatments interact with my Tamoxifen?

This is not known.

2. Are there any clinical trials available for women?

There are clinical trials, however inclusion is not dependent on gender.

  • Has there been any evidence of myositis affecting either heart or bowel muscles?

Yes, myositis can affect both heart and bowel muscles.

IBM: Research and treatment

  • With possible good results from the arimoclomol trial at the moment, what help and at what progression of IBM will it prove beneficial to later stage IBMers? Will it stop the progression or even help to regain what I’ve already lost with strength and mobility?

This is what the trial needs to prove.

  • Does IBM affect my heart, because heart is also a muscle?

No heart affects have been described in the literature, but I have five IBM patients, all of whom have elevated troponin T (a heart-specific muscle enzyme). Further testing (MRI, EKG), however, shows no pathology. However, since IBM patients are older (>60 years), the risk for heart problems is already increased. From my point of view, there is no extra need for concern for heart disease in IBM patients. There is only a laboratory hint, but no clinical evidence.

  • Is there any treatment or medication for IBM disease?

There is one clinical trial (arimoclomol) currently underway.

Sometimes intravenous immunoglobulin (IVIg) is used to slow disease course.

Until now, physiotherapy is the most helpful therapy.

  • New cancer treatments now include targeting specific genes and cells (immunotherapy) with some amazing results. Is that research applicable to addressing s-IBM?

Potentially yes, but this is unlikely at present. Targeted therapies are a major goal for myositis research.

Since the cause and mechanisms of the disease are less known and studied, we cannot answer whether immunotherapy will bring relief. Immunosuppression with prednisone and other medications does not help.

Dermatomyositis research and treatment

  • Are there certain things that trigger an episode with DM? If I knew, could I avoid the swelling and rash? I have suspected heat or sunshine. Perhaps antibiotics also.

Any infection can trigger a flare.

Heat or sunshine might worsen the symptoms in some cases.

Please avoid sun as it triggers the rash. When the rash is there, topical use of so called calcineurin inhibitors (e.g. pimecrolimus ointment) in addition to immunosuppressant therapy is helpful.

  • I have been diagnosed with dermatomyositis, and for a couple of years I have suffered from really painful calcinosis. Are there any treatment options of calcinosis?

Currently adequate immunosuppressive therapy is the best option. Unfortunately, this does not work well on calcinosis in many cases. It depends also on the size. For small (1-2mm) deposits, there are reports of successful use of a prescription containing sodium thiosulfate.

  • Should cancer screening be routinely part of the protocol for dermatomyositis?

Yes, especially when certain antibodies are found in the blood (NXP-2 and TIF-1).

Polymyositis research and treatment

  • Are there any new treatments for polymyositis, both newly diagnosed and cases that are resistant to treatment?   

There are many immunosuppressants used for rheumatic diseases that are currently being tested for such cases. 

  • Is there a new list of symptoms used to diagnose polymyositis? 

No, polymyositis is still diagnosed based on a typical pattern of muscle weakness along with muscle biopsy and other tests. Some researchers, however, are trying to avoid using the diagnosis of PM, feeling the diagnosis can be more precise by testing autoantibodies and other factors.

  • Are there new diagnostic criteria or specific treatments for polymyositis based on subtypes?

At this time there are no new or additional diagnostic criteria or treatments for polymyositis.

  • Are there natural treatment methods (alternative treatments)?

So far, no natural treatment has proven to have any substantial effect on autoimmune diseases.

  • I understand exercise is very good for polymyositis patients. I also can feel the benefits of exercise sometimes. I am in pain most of the time, especially around my shoulders and neck area. Should I continue to exercise, or I am making more damage to the muscles and joints in this area? Or, is there hope that the muscles/joints will improve with exercise and lead to reduction or no pain in the future?

The recommended intensity and the form of exercise depend on the activity of your PM. If there is more disease inflammation and you need a higher dose of corticosteroids, you have to be more careful and exercise with less effort and use a mild to moderate aerobic exercise. If your disease is more stable and you need a low dose of corticosteroids, you can be more active, perhaps even with a resistance training.

Necrotizing myopathy research and treatment

  • How is remission of HMA Co reductase (HMGCR) autoantigen necrotizing myopathy defined? Where do my CK levels and other inflammatory markers need to be at to say I am in remission?

Anti-HMGCR-myositis is thought to be autoantibody mediated. Remission is gauged by normal CK levels and no symptoms.

Remission should be measured clinically. Biomarkers can be helpful, such as CK or autoantibody titers. In anti-HMGCR-myositis, CK levels often stay elevated, and titers can correlate with clinical improvement.

  • With necrotizing myopathy, how many grams of protein, carbs, and fats should I shoot for daily? What is sane and realistic BMI for me? What about supplements?

Studies have not been done to identify nutrition-based treatment for myositis diseases, so this is not known. This should not make any difference, however, as the process is autoimmune.
 

  • I think I am right on the cutoff line for osteoporosis. What bone building formulas should I use for osteopenia?

Treatment is according to osteoporosis guidelines. For beginning osteoporosis, this includes vitamin D and calcium supplements.

  • What is a realistic bone building program for someone with osteoporosis and NM?

Standard physical therapy would be ideal.

Physical Therapy/Exercise

  • What exercises do you recommend for IBM?

Depending on the literature and my own experience, I recommend for IBM patients a combination of aerobic exercise and resistance training individually adapted. These exercise forms are very common and available. Overstrain should be avoided.

  • I was diagnosed with IBM about two years ago. I have noticed increased muscle burn when exercising, which I do regularly. If I push through the muscle pain, am I making my condition worse?

No, but you should not push it to the limit.

  • Can you gain back lost muscle thorough physical therapy?

Yes.

Getting back muscles lost cannot be expected. The aim must be to improve strength and functional capacity, stabilization of preserved muscles, and slowing down the process of muscle wasting and loss of strength.

General questions:

  • Recently, my doctor suggested he check my blood with an ANA test. The result is I am positive for polymyositis. What is the difference between “myositis” and polymyositis?

Poly just means “more than one muscle affected.”

There is no blood test saying you are positive for polymyositis. This is a diagnosis made by biopsy. I would recommend you consult a myositis specialist.

  • I have been told I will need prednisone for one year. Does myositis go away in one year?

No, myositis usually does not go away in one year. Autoimmune myositis is chronic, and treatment is based on symptoms and other finding.

  • Are there any type of myositis that presents with a drop head?

Not usually, but neck muscles can be affected, and drop head can develop during the course of the disease, but rarely. Drop head is typical for Sporadic late onset nemaline myopathy (SLONM), which is not myositis.

  • When a patient is pursuing a diagnosis of myositis, is there a recommended list of blood tests, including antibody tests, that should be undertaken?

We normally do CRP, CK, TSH, Na+, K+, Ca2+, ANA, anti-dsDNA (with typical symptoms for SLE), (pANCA, cANCA), myositis-associated antibodies, and myositis-specific antibodies.

  • Can the myositis physician community find better solutions than high dose, long term prednisone? Prednisone has been around for over 100 years and causes serious side effects.

There are multiple immunosuppressive options available as well as IVIg.

  • Has there been any research into possible genetic predispositions to myositis? I know that there is some talk about certain genetic mutations can lead to inflammation, which may cause autoimmunity problems. These then can manifest in various autoimmune diseases such as lupus, MS, psoriasis, and perhaps myositis diseases. Could there be any possibility of this?

In autoimmune disorders there are always genetic and environmental factors contributing to the pathogenesis. Genes might be risk factors but are not sufficient to initiate disease alone.

hIBM:

  • Is there a difference between treatment options being considered for those with genetic IBM vs those with sporadic IBM? If so, why are the options different?

At present there are no specific options, but for hIBM you can try sialic acid.

  • What is the newest information on familial inclusion body myositis? I have IBM, my mother had IBM, and I had a sister diagnosed with polymyositis. I also have an older brother who is not affected at all. Has any connection been found?

There should be a genetic but family-specific link to be diagnosed with fIBM.

  • Why is there not much interest with patients who are suffering with hereditary IBM (hIBM). We have a more rare condition yet need help as well. I feel we are the forgotten ones.

It’s not forgotten. It’s just that there are too many similar problems for the few experts. My suggestion is to go and see an expert for hIBM.

One comment on “Experts responded”

  1. 1
    Brenda Anderson on November 4, 2019

    Thanks for sharing.

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