Dr. Anthony Amato is the vice-chairman of the Department of Neurology and the Director of the Neuromuscular Division and Clinical Neurophysiology Laboratory at Brigham and Women's Hospital in Boston and a Professor of Neurology at Harvard Medical School. He is the chair-elect of the Neuromuscular Section of the American Academy of Neurology. He is the director of the Partners Neuromuscular Medicine fellowship program. His extensive research and published work reflects his long-time interest in DM, PM and IBM.
Ask a QuestionDr. Anthony Amato is the vice-chairman of the Department of Neurology and the Director of the Neuromuscular Division and Clinical Neurophysiology Laboratory at Brigham and Women's Hospital in Boston and a Professor of Neurology at Harvard Medical School. He is the director of the Partners Neuromuscular Medicine fellowship program. He researches and writes extensively about DM, PM and IBM, and will assume the chairmanship of the neuromuscular section of the American Academy of Neurology in April. Dr. Amato gave several presentations at the 2010 Annual Patient Conference in St. Louis. He has participated in TMA's discussions in the past. We welcome Dr. Amato back.
Dr. Amato:-
Cancer and Myositis
Participant:Good Day Doctor: We know Cancer can cause certain forms of myositis, can myositis such as classic dermatomyositis cause cancers (lung, colon, stomach, prostate, testicular, Ovarian, Breast, and Skin. Please elaborate. Thanks Wilbert
Dr. Amato:Cancer really does not CAUSE myosits and vice-versa, though they are ASSOCIATIONS with one another. It is thought that the body mounts an immune attack against cancer cells and some of the proteins on the cancer cells may resemble some proteins on muscle and skin. This leads to an innocent bystander attack on muscle/skin
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Heart Disease and PM
Participant:Good Day Doctor: Does PM and DM cause Heart Disease or does Heart Disease cause PM and DM? Please elaborate. Thanks Wilbert
Dr. Amato:Heart disease does not cause myositis. Rarely, the heart muscle can be affected by the inflammation.
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DM and ILD
Participant:Good Day Doctor: Does DM cause Institual Lung Disease (ILD) or does ILD cause DM? Please elaborate. Thanks Wilbert
Dr. Amato:Again, myositis does not cause ILD or vice-versa. There are associations with lung disease and cancer. Some patients get autoimmune attack against the lung in addition to muscle.
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Sun Exposure
Participant:Why are we advised to avoid sun exposure? Do we know at the cellular level what happens? Am I playing russian roulette by being in the sun without protection? (freckled red head, sunglasses, sun hat, moving into the shade when the sun gets to be too much.) Dx'd with pm 2002. Treatment began with 100mg daily of prednisone and 25 mg/ml of injectable methotrexate. Now no prednisone and 4mg/ml methotrexate, on the way to tapering to zero. One relapse 2006. Hypothyroid, Rosacea, reflux, approx. 65% strength and stamina return.
Dr. Amato:Sunlight can make rash of dermatomyositis worse. Too much direct sun is also bad because it increases risk of skin cancers which may be increased anyways because of the myositis and immunosuppressive medications. So one should always use sunscreen.
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PM and Anemia
Participant:I was diagnosed with autoimmune hemolitic anemia in 1974. Since 1977 I am not suffering from this disease .In 2007 I was diagnosed with PM. Is their any connection between the 2 diseases. Michael
Dr. Amato:There may be increase risk of other autoimmune diseases in patient with myositis, including hemolytic anemia.
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PM and CDM
Participant:Doctor thank you for addressing my following two questions. 1. I was diagnosed with both Polymyositis (PM) and Classic Dermatomyositis (CDM) and told my muscles are being attacked from my own white blood cells. My understanding is the CDM is causing white blood cells to attack my muscles indirectly via my arteries and veins, while PM is causing my white blood cells to attack my muscles directly. Can you elaborate on this? 2. Can people also have both PM and Inclusion Body Myositis (IBM) or CDM and IBM? If so please elaborate? Thank you
Dr. Amato:I doubt you have both DM and PM. If you have a classic rash, you probably just have DM. It is very unlikely also for someone to have DM or PM and then later develop IBM. More common is for patients to be mistakenely diagnosed as having PM when they really had IBM all along.
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Abtinuclear AB
Participant:I was diagnosed with PM in 2007 my Antinuclear AB was Positive 5.6. Now after 3 years my Antinuclear AB is 1.2 EQUIVOCAL . What is the meaning of this results. Michael
Dr. Amato:Means nothing. One does not usually follow antinuclear antibodies. I check them once at time of diagnosis as there may be increased incidence of an underlying connective tissue disorder in patients with these antibodies but they fluctuate all over the place and are not intended to be used to follow patents.
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IBM and Muscle Deterioration
Participant:I am a 80 yr. old female, I was diagnosed with IBM 4 years ago. I use a walker, drive my car, live alone and am independent. My right foot drops, toes on left foot curl, and am tired most of the time. I swim three times a week and have a social life. I have lost my hamstring muscle in right leg and quadracept muscle in left leg, notice the right leg has reduced in size. My question is whether any of the test being done at the Childrens Hospital in Columbus, Ohio have had any positive results? I take no prescription drugs, and enjoy good health except for IBM. Thank you, Audrey Gallagher
Dr. Amato:The follistatin study in Columbus has not started yet. It will begin hopefully in Jan/Feb 2011 or early spring.
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Gaining Muscle
Participant:I have chronic polymyositis. I desperately need to add muscle back into my body, especially my legs (quads). My knees are hyperextending due to the lack of muscle there and I cannot get any answers as how to accomplish this. Please help. Thank you, Deborah Ross
Dr. Amato:I wonder if you really have IBM based on your severe quadriceps weakness. What you describe is not typical of PM. Biopsy may not show vacuoles/inclusions in as many as 30% of IBM cases. Maybe you should see a physiatrist to assess if you may benefit from a knee brace.
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Muscle Weakness
Participant:Is the muscle weakness in PM always symetrical? And is PM always accompanied by ILD? I was first diagnosed with PM in 2004- now there is thinking that I have IBM- Have there been enough diagnostic advances since 04 to merit new diagnostic testing to try to clarify diagnosis?
Dr. Amato:Muscle weakness is typically symmetric in PM and often, but not always, asymmetric to some extent in IBM. ILD is seen in only 10% of PM. An experienced neuromuscular specialist can usually tell the difference between IBM and PM in at least two-thirds of cases.
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Multiple Systems?
Participant:I have been diagnosed with PM. Besides the typical symptoms, I have recently experienced multiple system involvement. Is this common? My gall bladder stopped working and had to be removed. They also think I have gastroparesis and cardiomyopathy. I also have ataxia and dyskinesia. Is this common?
Dr. Amato:None of these other symptoms are suggestive of PM. I would wonder about a mitochondrial neuromyopathy as opposed to PM based on the multisystemic problems you are having.
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Chloroform Exposure and PM
Participant:I was working for the past 32 years in a laboratory exposed to an excess of Chloroform. Could it be the excess of Chloroform i was exposed to, the cause for the PM i have. Michael PM
Dr. Amato:I don’t think so
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Excercise
Participant:I have had PM for 22 years Is there a website where PM patients can go to get exercises that we can do at home? What is your opinion regarding heredity in Myositis?
Dr. Amato:I am not aware of any website for exercises. Myositis is not hereditary. That said, the genes that increase risk for autoimmune disorders are inherited, so there may be a slightly increased risk of myositis and other autoimmune disorders in family members who have myositis.
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HGH for PM
Participant:Why doesn't Human growth hormone work for PM?
Dr. Amato:Does not effect the primary problem which is an autoimmune attack against the muscle
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IBM Fingers
Participant:IBM Why are my fingers so deformed with this awful disease? Why are fingers affected period? Some IBM people do not experience this condition. I can not bend them to pick up things. I am frustrated What can help this condition? Duaine
Dr. Amato:Great question and one we specialists wonder all the time ourselves. It is not known why certain muscle groups like the quadriceps in the legs and the finger flexors in the arms are predominantly affected in IBM. I would suggest a referral to an occupational therapist to see if there are any orthotic devices that may be of help.
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Ankles and Feet
Participant:IBM Numbness in ankles and feet, legs included. Can any thing resolve this condition? Any thing. How about physical therapy? What is your take Dr Amato? Thanks Duaine
Dr. Amato:They numbness is related to a polyneuropathy. There is increase incidence of polyneuropathy in patients with IBM but can be unrelated (usually related to age or diabetes). Unfortunately there is no treatment for the numbness.
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Tiredness
Participant:I have IBM, I seem to be tired all the time. Is there something that I can do about it? Thanks.
Dr. Amato:IBM won’t make you tired (example sleepy) but will make you fatigue easier. Nothing one can do other than preserving your energy (example, use a scooter instead of walking far distances if this is what gives you fatigue)
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Hereditary
Participant:I have IBM, diagnosed by biopsy 5 years ago. My mother died 6 years ago with all of my symptoms, and at 87 was about in the same condition I am at age 63. Do you agree with me that this is almost certainly a form of hereditary IBM? How can I be tested to find out?
Dr. Amato:Not necessarily. The pattern of weakness and muscle biopsy in hereditary inclusion body myopathy honestly usually look nothing like inclusion body myositis. Rarely, there can be other members in the family with inclusion body myositis, but this does not mean it is hereditary as I previously mentioned.
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Flu Shot
Participant:I am 83 with IBM which I have had for 19 years.Is there any harm in flu shots. I was told by family Dr. years ago, that it might affect the disease. I have never had one and luckily never had the flu. Also fingers almost useless now. What do you suggest?
Dr. Amato:No problem with flu shots- I actually would encourage getting the flu shots.
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Hammertoe
Participant:I am a 71 year old female. I was diagnosed with IBM in 2008. I have since developed a moderate hammertoe deformity on both feet. Is this condition associated with IBM? I am still walking and the hammertoes make it doubly difficult. Would an operation to straighten the toes aggravate the IBM?
Dr. Amato:Hammer toes are not common with IBM. Surgery would not aggravate the IBM
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Stem Cells
Participant:I was diagnosed with IBM 2 yrs ago. Predinsone does help me and now i'm on cellcept too in order to get off the predinsone in the hope that it will keep the benefits without the side-effects. Do you have any experience with Cellcept? Do you think stem cells will be the new drug for us? thank you for your time
Dr. Amato:I use CellCept a lot in PM and IBM but I have not found it to be effective in IBM. We are a long way from knowing if stem cells will be of any benefit
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Steroid Use
Participant:I have IBM could I use steroids to help me get more mussle mass?
Dr. Amato:Unfortunately, most studies have show the steroids do not significant increase strength in IBM. Rare patients though note a modest improvement in function.
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Remission?
Participant:Do you see many cases of remission (or symptom-free patients) several years after diagnosis in Dermato Myositis?
Dr. Amato:About 30% of DM may go into a remission- usually several years after diagnosis
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Vitamin D
Participant:I am a 77 year-old woman who has had DM for about 4 years. Not doing too bad; on cellcept. Right now, Vitamin D is really being hyped as some magic additive to our diet, and since my count was on the low side, I began taking supplements. Now, I read it boosts your immune system! Isn't that the LAST thing I want to do? Marian
Dr. Amato:The Vit D stuff on internet is a lot of hype without strong scientific evidence. That said, I often recommend vit D as it can help prevent loss of calcium and bone loss (osteoporosis) that increases with aging, lack of activity, and steroids. There is no evidence that taking vit D will actually worsen any type of myositis
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Hope of Remission
Participant:After over ten years of IBM is there any hope of remission.
Dr. Amato:IBM may slow down, but I am not sure there is any true remission per se
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Cure or Treatment for IBM
Participant:Is there a cure or treatment for inclusion body myositis using stem cell or other methods? Thankyou. Mike
Dr. Amato:Not at this time
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Plateau?
Participant:Is it possible to reach something like a 'plateau' in IBM where you seem to remain for a while in terms of your functional status. It seems to me that for the last year and a half that I am still at the same level as far as the IBM FRS is concerned. Or am I fooling myself?
Dr. Amato:Yes, IBM can progress so slowly that it may seem as if it has reached a plateau
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Exercise Treatment
Participant:What is the best exercise treatment for IBM Thank you Holly Zykwa
Dr. Amato:Range of motion and aerobic exercise (eg. Swimming)
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Hormone Therapy
Participant:I have DM. But have had prostate surgery and radiation treatments.Drs want to put me on hormone therapy because my psa has started to rise. With muscle weakness now this seems like a poor choice.Do you have any other suggestlons?
Dr. Amato:It is unlikely that the hormones would have any deleterious effect on your DM
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Neurologist
Participant:I am a 69 year old woman with IBM which was diagnosed 5 yrs ago by way of a muscle biopsy by a Plastic Surgeon. I have not seen my Neurologist since, but not for lack of asking both my Rheumy and GP to refer me back to him again. They seem to think I don't need this service, and say as much indirectly (of course!) My leg and arm muscles are certainly visibly deriorating and I am, naturally, getting weaker as time passes. I would like to know how I'm actually progressing with the IBM and feel my Neurologist would be the one to give me answers - I sure don't get them from the others. How often should patients see a Neurologist, yearly/bi-yearly or never again after diagnosis? I would be interested in your comments. With thanks. Gail MacDonald Ottawa, ON Canada
Dr. Amato:I usually follow patients with IBM every 6 months to a year
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Feeling better?
Participant:You diagnosed me earlier this year with IBM. I began falling down in a few weeks after diagnosisi. I have gone back on a regiman of high dose prednisone, methamextrate and immuran. i am feeling much stronger and better able to get around. If I have IBM, why are drugs working?
Dr. Amato:I am very glad that you are feeling better. The problem is that prednisone can make people “feel” better. When coming off prednisone. patients often “feel” worse. Prednisone can help with aches and pains associated with arthritis or muscle strain, so it may also help people get around better. There is also a placebo-effect associated with any drug as both the physician and the patients want to improve with treatments. I have taken care of many patients who “felt” they were stronger on medications, in whom, I was unable tell any difference when I examined them. It may be that this is the result of a placebo effect or that our abilities as physicians to detect mild degrees of improvement cannot be picked up on routine manual muscle strength testing that is routine done in the clinic. I cannot say that these medications have not contributed to an actual physical improvement in you or as I mentioned previously might slow the disease process down. However, the overwhelming experience from most specialists and from what is in the literature is that most (not necessarily all) patients, do not have a significant benefit from immunosuppressive therapies and the risks associated with these drugs often outweigh any benefit.
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Lead?
Participant:I'VE HAD IBM FOR 17 YRS.NOW,I THOUGHT I BECAME EFECTED FROM HANDLING INGOT LEAD FOR OVER 40 YRS. ANY THOUGHTS ON THE SUBJECT. THANK YOU MR.GUERRA
Dr. Amato:No evidence that lead can cause IBM
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Rising CK Level
Participant:Thank you for taking my question. I have IBM, 10 years now, I would like to know if rising CK level is OK and there is actually nothing can be done to stop it. Thanks, Enid
Dr. Amato:There is no reason to follow a CK level in IBM- it has no relationship to disease activity and is not something someone should treat.
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Scarred Muscle
Participant:I have had PM for over ten years and therefore I have a lot of scarred muscle and connective tissue in pelvic region. I understand this significantly prevents muscle regeneration. Is there any research in this needed area??
Dr. Amato:Great question. There are studies being done on medications that might help decrease scarring- mainly in the muscular dystrophies, but any advances here would potentially help with patients with scarring from chronic myositis.
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Prednisone
Participant:Do patients with polymyositis ever progress to taking no prednisone?
Dr. Amato:Yes, some patients with PM and DM can be weaned off prednisone and other immunosuppressive agents
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Diminishing Strength
Participant:I am 67 and was diagnosed with IBM in 2006. As my overall strength diminishes I feel the need to exercise more vigorously. How do I insure that my exercise is beneficial and not wasting more muscle? Is there some way to monitor this?
Dr. Amato:I generally recommend aerobic activities. You should avoid eccentric exercises- that is the slow lifting of weights and slow return to rest position. This stretches muscles while it contracts and tears them. While it is a way to quickly build muscle in healthy individuals, it breaks down muscle faster in patients with muscle disease. If you do lifting, it should be no more than 65% of you max and don’t do it to the point that you are exhausted and aching.
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Neuromuscular Clinic?
Participant:I am a 67 year old IBM patient. I live in a rural area and find that most Neurologist in my area don't have many IBM patients. Would it be any advantage to drive to a metropolitan area to be seen in a Neuromuscular clinic where they see more IBM patients?
Dr. Amato:I think it is best to be seen by clinicians familiar with your specific disease whenever possible. If the neurologist is not familiar with IBM, maybe there is a rheumatologist close by. Seeing a specialist though once a year or just once so they can advise your neurologist periodically is not a bad idea.
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Plasmapheresis
Participant:what is your experience with Plasmapheresis for DM they want to try it on me as no drug works on me also are there any new medicines coming out that are promising thank you david
Dr. Amato:I have not used plasmapheresis for DM. A small clinical trial showed it was not beneficial, but again the trial was very small so a modest benefit may have been missed. There are many new drugs that are being currently studied that may be beneficial in patients with DM and PM.
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PM and IBM
Participant:Is there any scientific basis that pm can develop into IBM or is the explanation that the original diagnosis was wrong?
Dr. Amato:No evidence that PM evolves into IBM. Rarely, it may be that the patient had two different dieases. However, the most common explanation is that the patient was initial was misdiagnosed as having PM instead of IBM. The reason is that most doctors don’t realize that inclusions are not seen in about 30% of any given muscle biopsy in patients with IBM. It is usually the clinical examination that is most helpful in distinguishing between IBM and PM but again, many physicians are aware of how to distinguish PM from IBM on the basis of the clinical exam.
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HGH and IBM
Participant:What, if any, experience have you had with Human Growth Hormone to help increase muscle mass in IBM? Can it be detrimental if used?
Dr. Amato:I have no experience with this. It certainly might be detrimental and I would recommended its use only in the context of a clinical trial in which one could actually objectively measure the risks versus the benefits
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Autologous Stem Cell Transplant
Participant:If IVIG is not effective in helping the dysphagia and continued degeneration, what about autologous stem cell transplantation as a treatment?
Dr. Amato:Stem cell treatment is purely in research stages at this time
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What is a mild case?
Participant:If a doctor tells says that you have mild IBM, does he/she mean that is the stage you are at NOW or does he/she mean that your case is a MILD CASE and may continue to be a MILD case?
Dr. Amato:You have to ask your doctor what it means, not sure but suspect he/she thinks your weakness is mild at this time
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Diagnosis Correct?
Participant:The rheumatologist keeps saying that my mom's bilateral peripheral neuropathy is unrelated to her polymyositis because her CPK values are WNL. Can that be the case?
Dr. Amato:Peripheral neuropathy has nothing to do with PM
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Exercise Benefits
Participant:I am age 79 with IBM confirmed in 2003. I exercised at health club on a regular basis until mid 2009 when use of a walker was required. To maintain my present mobility is use of 3 and 8 pound free weights and elastic bands at home appropriate exercise or would there be more benefit in the use of resistance machines and more weight at a health club.
Dr. Amato:As previously mentioned, I generally recommend aerobic activities. You should avoid eccentric exercises- that is the slow lifting of weights and slow return to rest position. This stretches muscles while it contracts and tears them. While it is a way to quickly build muscle in healthy individuals, it breaks down muscle faster in patients with muscle disease. If you do lifting, it should be no more than 65% of you max and don’t do it to the point that you are exhausted and aching.
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Remission without degeneration?
Participant:Male, 74 years old. IBM diagnosed by biopsy 12/06 (Dr. Harati – Houston). Apparently afflicted for several years prior. Dysphasia, no pain, no falling so far. Able to perform all routine daily life functions. Question #1: Does IBM ever just stop, or do affected muscles always continue to degenerate until patient becomes an invalid? Question #2: Are there sometimes prolonged periods of remission with IBM, when degeneration is not occurring? Thank you. T. H.
Dr. Amato:IBM can be so very slowly progressive that it may be difficult to tell if there is any worsening (e.g., remission)
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New Therapies?
Participant:At 74 yrs of age what are the realistic expectations that any newly discovered therapies will be available to me in my lifetime? I'm looking at the Ohio clinical trials with hope but with much skepticism as well. Charlie Baldwin PM IBM from 2006
Dr. Amato:I am an optimist and encouraged by all the recent advances in research over the past several years. So I hope that we will understand these diseases better and this will lead to better treatments in the near future.
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Follistatin
Participant:What is the status of the IBM gene therapy trial with follistatin?
Dr. Amato:The study is to start early next year
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Myostatin Inhibitor and IBM
Participant:Acceleron Pharma is in Phase II testing of its myostatin inhibitor ACE-031 on DMD patients. If it is successful, might this drug be applicable to IBM patients?
Dr. Amato:Great question again. I certainly hope so. I have already talked to them already about doing a trial in IBM.
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Knee Muscles
Participant:I am 87 years old with IBM and have one locking knee brace. will standing on vibrating plates aid me in retaining my diminishing leg muscles. Bob Cain
Dr. Amato:Not sure how this would help
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Mitochondrial Myopathy vs IBM
Participant:The doctors say I have conflicting symptoms: cannot rise from a chair, need a rail to climb stairs, weakness in hands, feet and legs, having these gradually worsening symptoms for ten years. I began in earnest in May 2010 to find out what's wrong. A lumbar and cervical MRI showed problems with L3-4, L4-5, C5-6 and C6-7. An EMG revealed chronic neurogenic denervation with moderate active myopathic findings. A muscle biopsy did not find inclusions or vacuoles (not done with an electron microscope), inflammatory myopathy with necrosis (no regenerating fibers), and a prominence of COX negative fibers, possibly suggesting a primary mitochondrial disorder. An EMS of the same biopsy has been ordered. First DX was polymyositis, but I did not respond to prednisone; PT did help. Now the DX may be IBM or gene problem. Question: What's the difference between IBM and mitochondrial myopathy. Is it possible to have both?
Dr. Amato:I suspect that you may well have IBM. As I mentioned previously, about 30% of patients with IBM do not have inclusions or vacuoles seen on any given biopsy. COX negative fibers are actually quite common in IBM (they also are more common with age). This suggests that stressors in IBM muscle probably also lead to increased disruption of mitochondria over time. Mitochondrial are an organelle in all cells that helps convert the food we eat into fuel/energy.
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Diagnosis
Participant:Trying to get a firm diagnosis these last five months has been frustrating and depressing to say the least. I've been searching for information on PM, IBM and mitochondrial myopathy. Do you have any book or Internet site recommendations?
Dr. Amato:I think the TMA website should be a good resource.
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Firm Diagnosis
Participant:I did not respond to prednisone for possible PM. Therefore, my rheumatologist would not prescribe methotrexate because of a suspected underlying primary mitochondrial disorder. In fact, she said she didn't think she could help me and I should find another doctor re mitochondrial disorders. I've been seeing a neurologist, and he's now scheduled me with a doctor at the Neuromuscular Clinic at Emory Hospital, Atlanta in FEBRUARY 2011. How important is getting a firm diagnosis quickly? Or, because there's no cure/treatment, what's the difference if its taking a year?
Dr. Amato:I don’t think that waiting until Feb 2011 is going to make a difference at this point
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Balance Issues
Participant:Does "trouble with balance come with this disorder?" Is there any cure or Trials in sight?
Dr. Amato:Sure, if the legs are weak there can be balance problems. There are trials underway for various types of myositis. I’m not sure that I would ever use the term “cure” at this time
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CPK Blood Test
Participant:The results of the CPK blood test indicate polymyositis, are there other signs we should watch out for? If so, what are they? Thanks, teresa
Dr. Amato:Results of CK tests DO NOT indicate PM. All an elevated CK tells anyone is that there may be some type of muscle damage going on. It does not tell anything about what is the actual disease. The signs that one should look for is weakness and it is the pattern of muscles that are affected that best help determine if a patient has PM, IBM, or something else.
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Gene Therapy
Participant:Has there been any further work on the Kaspar- Mendell Gene Therapy Research on Sporatic In-Body Myositis at Children's Research Institute in Columbus,OH? If not, what would be your best guess of when we might expect it to begin again? Thank you, Charles Jackson
Dr. Amato:Trial is suppose to begin in early 2011
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Exercise Recommendations
Participant:Three of my siblings (nine total) have been diagnosed with IBM. Currently our family is in a genetic research program at the NIH. My sister has difficulty with her hands and the muscle is "withering." Is blood circulation in areas impacted by IBM helped with medication or physical therapy. What are your recommendations for exercises?
Dr. Amato:It would be extremely unusual for 3/9 siblings to have typical inclusion body myositis. I wonder more about a hereditary inclusion body myopathy which is completely different. Regardless, circulation is not effected in either condition. I have addressed exercises previously.
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Hot Tub Usage?
Participant:59 year old male with IBM diagnosed one year ago. Is hot tub usages recommended or discouraged? Are there any "shots" or treatments available that increase muscle strength in the legs?
Dr. Amato:I’d just be careful with hot tubs so that you are not alone and can get out of the tub in there is any problem. There are no treatments at this time for IBM as discussed.
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Occlusion Training
Participant:After 6 mo. of PT my IBM symtoms are getting worse. Would occlusion training be appropriate?
Dr. Amato:I have never heard of “occlusion” training
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Muscle Weakness
Participant:why do muscles feel weakest after sitting, sleeping, or when you slow down?
Dr. Amato:Probably because when you sleeping or sitting you really out using them and then when you all of a sudden need to use them to arise from chair or bed you feel the weakness. Also, if one has arthritis, there is stiffness in the joints that worsens with inactivity and it may take a while to limber up.
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PM Diagnosis
Participant:I was diagnosed with PM in 2005. Initially, there was improvement with prednisone and methotexate, I began getting worse and in 2010 was diagnosed with IBM. Would it be beneficial to continue with prednisone and methotrexate? Thank you
Dr. Amato:If you are no longer improving (as is most often the case with IBM and immunotherapies), I’m not sure that continued use of these medications are worth the risks
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hIBM and sIBM
Participant:what characteristics distinguishes hIBM and sIBM? Can hIBM start late in life?
Dr. Amato:There are different types of h-IBM. There is one form caused by mutations in the GNE gene that usually starts before the age of 30 yrs with progressive foot drop. The quadriceps are relatively spared. Another type of h-IBM cased by mutations in VCP gene can occur at any age, even late adult life and can affect the hips and shoulders or more distal hand and foot muscles. This type may be associated with Paget disease of the bone and dementia.
sIBM almost always starts after the age of 40 (usually after 50) and two-thirds of patients manifest with weakness in finger flexors in the arms and the quadriceps in the legs.
The biopsies in sIBM show inflammation, while there is little inflammation in h-IBM
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Describing Rare Diseases
Participant:Since PM and IBM are considered rare diseases, I find most people have never heard of them. How do I best describe these diseases in a general way to family and friends; are these accurate statements?: "I have a muscle problem." "For some reason, my immune system is attacking and destroying my muscle cells." "PM has treatments, but IBM has no treatment or cure." "IBM is like ALS, only slower."
Dr. Amato:All are reasonable terms to describe IBM except I would not say it is like ALS because it is really much, much slower and does not usually affect life expectancy.
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Gene Testing
Participant:What's your opinion on gene testing to determine possibility of heredity? Perhaps my children and grandchildren need this information.
Dr. Amato:One would not do gene testing if pattern of muscle weakness and muscle biopsy looks like sIBM as opposed to h-IBM
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Muscle Weakness
Participant:Realizing that every IBM case is different, generally speaking does the muscle weakness progression rate remain consistent, or does the rate of progression vary? For example, if I'm at a certain stage after ten years, could I expect the muscle weakness to double in another ten years?
Dr. Amato:It is not clear as there has not been natural history trials beyond a year or so. In my experience it is slowly progressive. I have never seen it suddenly worsen at a faster rate, though it can seen to slow down or plateau.
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PMR and PM or IBM
Participant:I had polymyalgia rheumatica (PMR) six years ago. I am currently in the process of getting a firm DX of either PM or IBM. Is PMR an indicator for either PM or IBM?
Dr. Amato:PMR is unrelated to PM, DM, or IBM
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Involuntary Muscles
Participant:PM and IBM attack voluntary muscles. Do they ever affect involuntary muscles, such as bowels, intestines, etc.?
Dr. Amato:No
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Hammertoes
Participant:Are hammertoes usually found in IBM patients?
Dr. Amato:Not typically associated with IBM
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Transfer factor
Participant:IS THERE ANY BENEFIT FOR TAKING TRANSER FACTOR? I LIKE MANY OF US IBM PATIENTS AM TRYING TO MAINTAIN STRENGTH. THANK YOU, TIM (TMA MEMBER)E
Dr. Amato:I have never heard of “transfer factor”
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Testosterone
Participant:Testosterone is known to enhance muscle development in athletes. Has it been considered to build muscle mass for PM or IBM in women?
Dr. Amato:Anabolic steroids have been tried and have shown no effect
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Gene therapy
Participant:What is the probability for there being gene therapy or other effective treatment for sIBM within the next five years?
Dr. Amato:I am hopeful
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Rheumatologist or Neurologist
Participant:Who should be the primary specialist for a patient with PM or IBM: a rheumatologist or a neurologist?
Dr. Amato:It really does not matter as long as the specialist is familiar with the disease
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Creatine
Participant:Somewhere I read taking creatine helps with PM/IBM muscle weakness but the patient must exercise rigorously and regularly. Is this correct?
Dr. Amato:There is no good scientific evidence that creatine is helpful in myositis
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Alzheimer
Participant:"Alzheimer characteristic proteins" are also associated with IBM patients. Does this mean that IBM patients are at an increased risk to develop Alzheimers?
Dr. Amato:There is no evidence of an increased risk of Alzheimers in IBM. In fact, I have never seen a patient with IBM and dementia.
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Rating Scale
Participant:I read your article on TMA's site (click "Research," then "Published Research," then "Inclusion Body Myositis" to reach Dr. Amato's article entitled "Inclusion Body Myositis: Old and New Concepts"). The IBM Functional Rating Scale (and the photos showing atrophy of both forearms) helped me to determine how I'm doing (I'm 32 out of a possible 40.) Are there more PM/IBM rating scales a patient can access in order to rate where he/she is in the progression of these diseases?
Dr. Amato:This is the only scale out there specific for myositis at this time, but we are looking at devising other scoring systems as current ones we use (eg. Manual muscle testing in the office) may not be sensitive enough to detect modest changes (improvements or worsening)
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COX Negative Fibers
Participant:What's the significance of "a prominence of COX negative fibers" in a muscle biopsy?
Dr. Amato:This is an indicator of mitochondrial damage. It is something everyone may get on biopsy as they age but is more frequent in IBM muscle. It suggests that whatever causes IBM or the stressors in muscle fibers in IBM may also lead to mitochondrial damage
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Hereditary IBM
Participant:Do I have this correct???: the hereditary form of IBM can only be present if there is no accompanying inflammation.
Dr. Amato:That is correct
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HGH and Steriods
Participant:Can human growth hormone be used in conjunction with steroid treatment to build muscle in PM/IBM?
Dr. Amato:not been tried
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Antivirals and PM/IBM
Participant:We are learning more about retroviruses present in chronic fatigue syndrome and fibromyalgia. Have antivirals been used to treat PM/IBM?
Dr. Amato:no one has ever demonstrated any evidence of an active viral infect in any of the myositides- so no
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Paget Disease
Participant:Should IBM patients with osteoporosis also be tested for Paget Disease, especially since there appears to be a genetic risk and possible association with dementia?
Dr. Amato:the hereditary inclusion body myopathy associated with PD and dementia is completely different then more typical sporadic inclusion body myositis, so the short answer is no.
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Aching Hands/Thighs
Participant:I am a 60 year old woman and was diagonosed with IBM two years ago. At the moment my only significant symptom is dysphagia. Question: I have been experiencing tightness/aches in my thighs, hands and fingers lately. Are these symptoms associated with IBM?
Dr. Amato:They could be
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Swallowing Issues
Participant:Background: I am a 60 year old woman diganosed with IBM two years ago and dysphagia is my biggest symptom. I have been having swallowing issues for nearly 9 years which have worsened over time. Question: What suggestions do you have to help with day-to-day severe swallowing issues?
Dr. Amato:I would suggest seeing a speech therapist who specializes in swallowing problems and getting a videoswallow test. There are many techniques that can be taught to you to help in swallowing. If this does not help one can sometimes have a dilation procedure done or surgery to help with swallowing
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Dysphagia
Participant:60 year old woman diagnosed with IBM 2 years ago. Most significant symptom is dysphagia. Question: Do you believe exercise is helpful to IBM patients for both the short and longer term? (I currently do gentle yoga 2x a week and work out with a personal trainer 1 - 2x a week focusing on muscle strengthening and conditioning.)
Dr. Amato:I would not work with a personal trainer doing intense exercises to build strength- this might damage muscle as I discussed. But yoga, range of motion, aerobic activities, or mild exercise is good
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CoQ10
Participant:Do you believe CoQ10 is helpful for IBM patients? It was suggested by my doctor to take between 800 - 1,000 mg/per day. I took it for about 1 1/2 years and then stopped because I did not feel any difference.
Dr. Amato:No evidence that it would be of any help
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IVIG
Participant:I feel no direct benefits from my IVIG treatments. They do not impact my CPK score. Are there indirect benefits to IVIG that I should be aware of?
Dr. Amato:CPK levels are really meaningless to follow in IBM. There have been several controlled studies that have shown that IVIG has no effect in IBM (if this is what you have). If it is not improving strength there is no reason to continue
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Improved CPK Levels and Tiredness
Participant:My CPK levels are excellent for over a year now but I still feel weak and tired after the littlest of activity, why?
Dr. Amato:I don’t know what disease you have but CK can be normal in DM and IBM. It may be that your muscles are also weak from damage that was done in the past or related to atrophy from chronic steroids or disuse
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Standards for Prednisone
Participant:I have been suffering with polymyositis since January 2007 and have been off Prednisone for several months. I still take 17.5mg. Methotrexate orally once a week. My question is when is a flair deemed a flair? When is muscle damage occuring? My CPK has risen to 700 but my strength seems unaffected. Should we have standards around when its time to prescribe Pred. or not ?
Dr. Amato:I usually treat the patient and not the CK level
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ACE031
Participant:Do you expect ACE031 to become a treatment for IBM any time soon, and will it be safe.
Dr. Amato:I have no idea
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Arimoclomal
Participant:Do you expect arimoclomal to become a treatment for IBM any time soon and would it be safe.
Dr. Amato:I have no idea
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Safe Treatment
Participant:What kind of time frame do you expect before there are safe effective treatments for IBM?
Dr. Amato:not sure
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Hair Loss
Participant:Is hair loss ever associated with PM or IBM if one has never been on methotrexate and only on a 6 week trial treatment of prednisone (which didn't work)?
Dr. Amato:Hair loss can be associated with PM (autoimmune alopecia) or related to treatment such as methotrexate. No relationship to IBM.
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Immune-mediated myopathy?
Participant:I'm confused: Is immune-mediated myopathy a sub-class of PM/IBM or is PM/IBM a sub-class of immune-mediated myopathy? Please clarify.
Dr. Amato:M is a type of immune-mediated myopathy.
We don’t know for sure if IBM is immune-mediated or a primary degenerative disorder of muscle
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Muscle Biopsy Site
Participant:How critical is the site of the muscle biopsy in determining PM or IBM? Is a second biopsy recommended if, for example, the first biopsy was of an upper thigh muscle and not of the weakened quadricep?
Dr. Amato:Muscle biopsy is essential in combination with a good exam in distinguishing PM from other disorders it may mimic. I don’t often repeat a biopsy as I can usually tell if a patient has IBM by exam
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Responsibility for Biopsy?
Participant:Communication can break down when there are several specialists involved. When a muscle biopsy is needed, whose responsibility is it to select the proper biopsy site and ensure that appropriate laboratory tests are done: the rheumatologist, neurologist, surgeon, patient?
Dr. Amato:The surgeon is only going to do what the referring doc asks. It should be a the direction of the specialist who is ordering the biopsy to select the proper muscle
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Indicators for IBM
Participant:Are there indicators or a test to determine if facial muscles are involved in IBM?
Dr. Amato:I usually just ask the patient to close their eyes real tight and see if i can open them
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Dysphagia Indicators
Participant:Are there indicators or a test to determine if dysphagia is involved in IBM?
Dr. Amato:yes, a video swallow study can be done
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Lipoatrophy
Participant:How often in an adult do you see lipoatrophy with dermatomyositis? What is the significance of this?
Dr. Amato:I have not seen this that often
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Weight Training
Participant:will weight training help or hinder IBM?
Dr. Amato:I would not do heavy, slow weight lifting as it can damage muscles as I previously discussed
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MTX and DM
Participant:I take MTX for my DM. The MTX has worked wonders on the muscle inflammation and my ILD, however, the skin part of the disease is not under control. What do you recommend for the itching and red patches?
Dr. Amato:The skin activity does not always follow muscle activity and vice-versa. I would try topical creams to start and if this does not work, try plaquenil
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Fallistatin
Participant:What is the status of the Fallistatin clinical trial?
Dr. Amato:will start in early 2011
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Diagnosis Code
Participant:What is the official diagnosis code when you display both PM and DM. A biopsy confirmed PM but the doctor said I also have DM. This is an important issue when hospitalization is required. The diagnosis code determined the number days stay you are alotted.
Dr. Amato:you can’t have both DM and PM
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IBM and Dementia
Participant:Does IBM have a connection to the onset of dementia?
Dr. Amato:no
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IBM Triggers
Participant:What is the current understanding of the nature of IBM (inflammatory or not, etc) and how is it triggered?
Dr. Amato:it is still unclear and controversial
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IVIG for IBM
Participant:I am getting IVIG for IBM. Has it been effective in limiting the progression of the atrophy?
Dr. Amato:No
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Self-limiting?
Participant:Good Afternoon, My Dr. mentioned that some physicians feel IBM is self-limiting in some instances. What are your thoughts on this. I was diagnosed in 2007. Marisa
Dr. Amato:It is usually slowly progressive
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Difficult Diagnosis
Participant:I seem to be one of those cases where it has been very challenging to determine whether I have PM or IBM. Do you have a suggestion other than taking a wait and watch approach.
Dr. Amato:Usually an experienced neuromuscular expert can tell the difference based on the clinical examination and biopsy
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Water aerobics>
Participant:I have IBM and in my water aerobics class do pull ups (60) under the diving boards which have seemed to help my lungs and asthma. Would this kind of exercise be wrong for IBM?
Dr. Amato:No, I think water aerobics is a good activity
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Genetic Predisposition
Participant:If a genetic predisposition is the underlying cause of pm is there any understanding of what triggers the disease?
Dr. Amato:Some wonder if an exposure to a virus in the past coupled with inheritance of a variety of genes may predispose to development of different types of autoimmune disease, including DM and PM (perhaps also IBM)
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Physical Therapy for Pain
Participant:Why isn't there a greater emphasis on physical therapy to treat severe cases of pm and dm? It seems like many treating doctors neglect this aspect of treatment of their patients.
Dr. Amato:Don’t know but I always refer to Physical therapy / physiatry
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Methotrexate
Participant:Do/would you ever try methotrexate w/IBM patients, and if yes, how long do/would you wait to see if there seems to be any effect (objective or subjective)? It's a long shot I'm willing to take, but I've only been on it for six weeks. Thank you
Dr. Amato:There has been one controlled study of methotrexate in IBM that showed it did not significantly improve strength. I don’t know if it may slow the progression down or not. If patients understand the risks, and want to try it, i may sometimes give a short trial of 3-6 months.
This has been another GREAT discussion. Thanks to all the members who participated today. And a special thanks to Dr. Amato for being with us and graciously spending the time to answer your questions.