on April 01, 2016
We are pleased today to welcome Dr. Mazen Dimachkie, a member of TMA’s medical advisory board. Dr. Dimachkie, a neurologist, is the director of the Neuromuscular Section at the University of Kansas Medical Center. He also serves as the director of the Electromyography Laboratory and two fellowship training programs in Clinical Neurophysiology and in Neuromuscular Medicine.
Dr. Dimachkie, thanks for joining us today.
Mazen Dimachkie, MD: I am delighted to join this event.
TMA Member: I have heard about a new drug to treat IBM called arimoclonol. What can you tell me about it? Will there be any more human trials?
Mazen Dimachkie, MD: We found the drug to be safe in a small pilot study. We are planning a larger study of arimoclomol in IBM, so please stay tuned! Best, MMD.
TMA Member: I was diagnosed with PM in 2012 and was on prednisone + azathioprine therapy for 2 years. Under my doctor’s supervision, I tapered off both meds and did fine for 18 months or so, at which time I had become significantly weakened and found that I had recurred. I was started on prednisone + azathioprine again. This time, I found that Medicare would not pay for the azathioprine (they did in 2012-13), stating it was not an approved drug for PM. It appears that prednisone and methotrexate are the only oral approved therapies for PM. Is there anyone who would help patients advocate for coverage of azathioprine for PM? I can’t afford to pay $150/month for this medication out of pocket. What are my other options for recurrent PM?
Mazen Dimachkie, MD: Your treating physician can send a letter of appeal that references the articles on azathioprine in polymyositis. Best, MMD
TMA Member: PM, 53 year old female: I have been diagnosed with fibromyalgia since 2012 and then in 2015 because of more severe pain, weakness and balance issues I underwent muscle biopsy from the thigh and MRI of the same thigh both confirming polymyositis. My CPK is always within normal range or just barely above normal. I have tried prednisone for 3 months with no help, Plaquenil for 3 months with no help and now on Imuran for one month with no improvement yet.
With a normal CPK since day 1, I feel like the “odd man out” as far as how my rheumatologist is approaching the treatment because I am not “severe” enough with my symptoms. I just know that my body hurts every day all day and my fatigue is making me nonfunctional. A lot of other testing has been done to rule out other diseases. At what point is IBM discussed or decided for a final diagnosis?
Mazen Dimachkie, MD: IBM is quite different from polymyositis. The pattern of weakness in IBM is quite distinctive. It is odd for polymyositis to not have a significantly elevated CPK. Best, MMD
TMA Member: My 32 year old niece that live in Trinidad & Tobago was diagnosed with Polymyositis about three years ago. It has progressed to the point that she is unable to move any part of her body except her head and fingers. Her neck also has gotten weak and she usually use a neck brace. Is it unusual for this to happen? Are there any international resources that can help her? We are so desperate.
Mazen Dimachkie, MD: I truly feel for your difficulties with this delicate situation. I wish I knew someone there to direct you to. Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: Doctor – I was diagnosed with Polymyositis 5 years ago (biopsy). After 5 months on high dose steroids, I started with fractures in my spine. Fortunately, it was determined that I was in remission as I am to date. I was weaned off the steroids but the damage was done. Over the past 5 years I have broken 9 vertebrae…I am now on prolia and have been pleased with the bone growth. Because of the fractures, physical therapy has been sketchy….difficult to build done back up when simply picking up something from the floor fractures another bone. I am not undergoing P.T again and am hoping to build muscles back…still cannot lift my arms and I have continued problems with my neck. Is this common problem…does this seem to be a problem that I might be facing for the rest of my life? Is there hope for me? Thank you.
Mazen Dimachkie, MD: Bone fractures and osteoporosis are some of the known complications of chronic steroid therapy. I usually refer my patients to a bone endocrinologist. Best wishes, MMD
TMA Member: After being initially diagnosed in August 2013, I was able to largely resume normal life (with limitations) for the remainder of that year. Unfortunately, in January 2014 I started experiencing lung involvement that completely flipped my quality of life. Within a week, I had three experiences of feeling that my lungs had suddenly been ‘capped’ in every direction, no longer able to expand. It was startling, and this new baseline has changed my life ever since.
A frustrating a note to this is that it was initially difficult to explain to urgent care or ER doctors that I the physiological issue came first, and hence I was becoming nervous about my breathing…rather than being a sort of panic attack.
The cautious nature of ‘losing my breath’ has made me unreliable to get to class or work. I have not travelled since December 2013 and am having trouble planning my wedding day. While terrible, the skin and muscle involvement I could battle while still living my life largely. With the breathing I cannot.
I can’t stress how much my quality of life would rise with a solution to shortness of breath. My CPAP helps at times, perhaps because it is helping in the push to move air. I do not currently have signs of methotrexate toxicity, or ILD.
My question is..
Do patients ever bounce back from this? What techniques or treatments are being used to combat air hunger, and maybe allow the mind to gain more trust in the body to reenter the world?
Mazen Dimachkie, MD: It is important to consult with a pulmonary specialist familiar with myositis on issues regarding breathing. Myositis is at times associated with interstitial lung disease which can be measured based on either a CAT scan of the chest or pulmonary function testing. There are some drugs that work better than others for interstitial lung disease. Consulting with an expert is my recommendation. Thank you for your inquiry, MMD
TMA Member: There has been some buzz on the Internet recently about Arimoclomol. I believe you were involved in an earlier clinical trial. Is there enough promise to continue to study it and does it hold any promise for PM?
Mazen Dimachkie, MD: I am not aware of any study of arimoclomol in PM. We are planning a larger study of arimoclomol in IBM, so please stay tuned! Thank you for your inquiry, MMD
TMA Member: Dear Dr. Dimachkie:My name is Monica Mayer, and I was diagnosed with Dermatomyositis on April 16th, 2014. As a current member of The Myositis Association, I thought I would share a bit of my story with you and the results I’ve had. I’ve tried medication prescribed by my Rheumatologist, particularly Methotrexate and Prednisone. Later, I made the decision to stop them since an ultrasound showed my liver was enlarged and enzymes were out of normal range. I’m choosing functional medicine for now, however the Dermatomyositis is not in remission just yet. My niece Patricia suggested I try Liquid Biocell by Jusuru (www.agedefyingscience.com/patriciapavon). This product is a naturally derived matrix of liquid collagen, hyaluronic acid, and chondroitin sulfate, and is manufactured into a unique patented molecular weight that makes it highly bioavailable to the body. After 5 weeks, I have noticed remarkable improvement in my joints especially my hands, back, neck, and knees.If you could please review this product and let us know your opinion, I would greatly appreciate it. Upon your approval, I would like to share the information with my fellow myositis members. Thank you so much for your time. In search for a better quality of life!
Mazen Dimachkie, MD: There are many products out there with claims to treat different diseases. I generally caution my patients about safety of these agents and their cost. Promising drugs should be tested in clinical trials in order to identify their role in treating myositis. Thank you for your inquiry, MMD
TMA Member: I have trouble understanding what my prognosis with PM really is. I had a very severe case in the beginning (10 years ago) and, after massive doses of prednisone and some other drugs have been fairly stable, with occasional flares (2-3 over the years) that are quickly treated with prednisone. I am 79 and haven’t had a flare for 5 years. So two questions: What symptoms should I look out for as I get older? Am I likely to be disabled? Thanks for your interest in myositis! What should I be aware of?
Mazen Dimachkie, MD: If a patient has normal strength, normal muscle enzyme, normal lungs and no skin manifestations of myositis this is a good state. Best wishes, MMD
TMA Member: What is the frequency of getting an ILD condition after a PM diagnosis. I’ve recently had a number of lung infections that are more acute than ILD but caused by an artificially suppressed immune system.
Mazen Dimachkie, MD: 5 to 10% of patients with myositis develop ILD. Best, MMD
TMA Member: I was diagnosed with PM 10+ yrs ago. I belong to a Myositis Support Group and people often respond that they have PM and DM. Is this possible? I was told by my Neurologist that you could not and that the muscle fibers from biopsy will determine what type you have. Thank you for your answer.
Mazen Dimachkie, MD: It is usually one or the other. Best, MMD
TMA Member: I am a PM patient and also have plantar fasciitis and oliotibial band syndrome. I would like to seek treatment for both these painful conditions but I am afraid the medications might interact badly with my prednisone regimen. What would you recommend?
Mazen Dimachkie, MD: Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: I saw you were a co-author of a study that found that new PM patients were more likely to die from infection than anything else. What infections are the most common? How can we prevent them?
Mazen Dimachkie, MD: It was a variety of infections. It is important to seek medical attention promptly if you develop any symptom of infection. Best, MMD
TMA Member: I have a fairly uncomplicated case of PM and am now treated by my family doctor as the closest rheumatologist is so far away. I seldom have flares but when I do they seem to be well controlled by prednisone. I would like to know what to expect in the future. How and where can I get my antibodies tested to see if I am at risk for lung disease.
Mazen Dimachkie, MD: Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: I wonder if I could be misdiagnosed. I have had PM for 20 years, but have never responded to treatment. I am slowly getting weaker and have trouble going up and down stairs. How can I find out if I might have IBM?
Mazen Dimachkie, MD: Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: I would like to ask a question about IBM. I am a 73 year old male IBM patient. I read about your work with Arimoclomol in rats. How has Arimoclomol done in human subjects? Thanks for your work with myositis patients.
Mazen Dimachkie, MD: We found the drug to be safe in a small human pilot study. We are planning a larger study of arimoclomol in IBM, so please stay tuned! Best, MMD
TMA Member: I am having a difficult time getting an accurate diagnosis. Recent lab work from RDL Laboratory shows I have an ANA Titer of 1:160 speckled patterns as well as a weak positive PM/SCL. My ESR and CRP tests are consistently elevated. I experience wide spread muscle aches, fatigue and myalgia. Since my blood work doesn’t show muscle inflammation, the specialist says I don’t have polymyosotis or overlap syndrome. They are calling it UCTD. An accurate diagnosis is important to me before I start taking prescriptions for my symptoms. Any advice or suggestions would be most appreciated!
TMA Member: Is it common in PM to have neurpathy (no diabetes). – affects feet and hands. How about IGM / mcus – bone marrow biopsy was negative of issues – can problem be in the future?
Mazen Dimachkie, MD: Peripheral neuropathy is a common problem that affects 3 to 7% of the US population. There’s no relationship between polymyositis and neuropathy. MGUS risk also increases with aging and can be seen an otherwise normal people. Please consult with your hematologist regarding follow up of MGUS. Thank you for your question, MMD
TMA Member: Hello Dr. Dimachkie, My name is Fay Stancliff and I was dx’d with PM & ILD and anit Jo1 antibodies in 2004, at the beginning of this battle my cpk was 4400 today it ranges around 74, I am only on a maintenance dose of Imuran 75mg and have been in a drug induced remission since 2010, my question is will I ever be able to get totally off Imuran? There have been times when the Mechanics hands flair and the only thing that seem to calm it is increase of Imuran to 125. Is there any test to determine if the Myositis is gone? I was told by a neurologist at one point that the PM will likely burn itself out! Is this a possibility? Thank you!
Mazen Dimachkie, MD: In general, if a patient has normal strength, normal muscle enzyme, normal lungs and no skin manifestations of myositis, remission is suggested. However, most myositis patients, but not all, require chronic treatment. Best wishes, MMD
TMA Member: Is it recommended to seek medical advice from John Hopkins or Mayo Clinic who have Myositis center vs relying on Rhuem. in area due to unique issues of PM/DM – myositis in general? in seeking a Rhuem. very few have PM listed on their list of specialities. Also what is the number of people known today with PM?
TMA Member: Have there been any improvements in MRI for detecting active polymyositis? Having undergone 2 muscle biopsies, I want to determine if my condition has again become active (off meds since 10/15) and would prefer and MRI.
Mazen Dimachkie, MD: MRI can be useful as is strength, CK and EMG. Best, MMD
TMA Member: I have PM with ILD. I am doing physical therapy and have noticed minor improvement in muscle strength but no increase in muscle mass. I have lost 35-40 lbs of muscle mass. Will imunnosupressants stop my muscles from deteriorating or will my muscles always deteriorate but at various rates based on the strength of my immune system as indicated by the associated blood tests for inflamation, muscle enzymes etc.
Mazen Dimachkie, MD: Imunnosupressants stop muscle wasting in PM and DM. Best, MMD
TMA Member: At what point in the disease process do you recommend a muscle biopsy? I have been diagnosed through a skin biopsy to have dermatomyositis. Progressing more into muscle now. I wonder if I should consider biopsy to make sure its not IBM. How quickly can this progress? I have been on Methotrexate for 2 years now, helped skin condition, but my joints/muscle failing. Got off prednisone, August 2015, due to weight gain and pre-diabetic state. Maria (same person that asked about knee replacement).
Mazen Dimachkie, MD: Please consult The Myositis Association website for the closest expert. Best wishes, MMD
TMA Member: Is it recommended to have a knee replacement when you are suffering from this disease? I am a 2012 breast cancer survivor, who has major joint/muscle issues. In May, 2015 on my right knee I was told I neeed a knee replacement. Arthroscopic surgery was performed. In October, 2015 on my left knee I had arthroscopic surgery for much of same issues. Last Saturday pulling weeds in my flower beds, caused my left arm to be majorly aggravated, I can’t get my lymphedema down now at all. And I am dealing with very limited mobility and severe pain in my shoulder and arm. Much of what my knees and my past experience with them have been.
I recently went to new rheumatologist, March 24, for numerous reasons and the fact that I am declining considerably fast. I have shooting pains throughout my body which he wonders if Fibromyalgia is not part of. I need advice on whether I should attempt this replacement. I feel my body is so frail and wonder if it will really solve any issues or just complicate things worse. Please, if you could give me your opinion it would help tremendously on which way to go or what further questions to ask.
TMA Member: I was diagnosed with PM 15 years ago but now have been told I have Immune Mediated Necrotizing Myopathy with anti-SRP. Apparently this new diagnosis is being found more and more. What are the treatment implications and is the prognosis any more positive than initially thought? (I just started getting Rituxan infusions). Thank you.
TMA Member: Hi Dr. Dimachkie, I was wondering how can one be diagnosed with PM by Muscle Biopsy then a few years later find out you were misdiagnosed and have either DM or IBM? Thank you,
Mazen Dimachkie, MD: It is not unusual to get a dx of PM which is later on revised to IBM.Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: Hello Dr. Dimachkie- I have had Polymyositis for over 15 years, diagnosed in my twenties. I had a severe case and have been resistant to many drugs over the years. I am now disabled (using a walker) but my CPK is normal. I was told by numerous doctors that I will not be able to gain muscle strength as the muscles are degenerative. Despite going to PT and aqua therapy, I have seen a continual decline in strength. Why would I still be losing strength if my CPK is normal? Could it be that my diagnosis has changed to IBM? Lastly, do you see any promising new treatments on the horizon for regeneration of muscle strength in PM? Thank you.
Mazen Dimachkie, MD: You pose interesting questions. Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: What is the best/easiest way to explain what Polymyositis is to someone that does not understand?
Mazen Dimachkie, MD: Myositis is muscle inflammation triggered by the immune system attacking your own muscles. Best, MMD
TMA Member: What percentage of those people who are diagnosed with myositis achieve full remission? What factors seem to affect whether full remission is achieved or not?
Mazen Dimachkie, MD: Up to 20% of myositis patients achieve remission. Factors predicting that are not well established. This area needs more studies. Thank you for your interest, MMD
TMA Member: Are the cardiac conditions associated with myositis usually more specific or can it be anything? For example, about 10 years ago I had a normal echo, I started showing signs of DM about 4 – 5 years ago, I had a repeat echo 4 months ago that shows the tricuspid, mitral, and pulmonary values all have regurgitation now. My EF has dropped off but is still WNL at 55%. Is this specific to DM or my overlap of lupus? Luck of the draw?
Mazen Dimachkie, MD: Luck of the draw! Hang in there, MMD
TMA Member: Dr. Dimachkie, I believe my disease is well treated and I am fairly able to live an active life. However, whenever I ask my rheumatologist what I can be doing to improve my health, he just says to keep taking my meds. Is there anything in lifestyle changes that will improve my future health. My family doctor says that losing weight will help me stay mobile longer and help my respiratory symptoms. What do you think?
Mazen Dimachkie, MD: Being overweight is not generally good for your health. Best wishes, MMD
TMA Member: I am a 39 year old female. Years ago, a rheumatologist told me that I have both DM and PM. My present physician said this is no longer considered possible. I’ve never had skin symptoms even during flares. What do you think about this diagnosis?
Mazen Dimachkie, MD: It is usually one or the other. Please consult The Myositis Association website for the closest expert. Best, MMD
TMA Member: Dr. Dimachkie, my rheumatologist told me that PM might be many different autoimmune diseases whose features are subtly different but all causing weakness. Is any research being done on this? Will PM eventually be treated on a more individualized basis? Thanks for your help.
Mazen Dimachkie, MD: There is a lot of ongoing research in myositis. So please keep checking TMA website. Best wishes, MMD
TMA Member: Dr. Dimachkie, I am a 44 year old female. Although I had a bad response to prednisone in terms of side effects, it controlled my muscle weakness very well. My family doctor suggested that I try a no-salt, no-flour,no sugar diet while on prednisone and I was amazed to see that it helped my water retention, weight gain and even seemed to reduce my mood swings and sleeplessness. Is there an explanation for this?
Mazen Dimachkie, MD: The low calories / low salt diet helps control swelling and weight gain. Best, MMD
TMA Member: Dr. Dimachkie, I have PM and it is never quite in remission, although I live a fairly normal life. I tire easily. Lately, I have been working out with some intensity and walking four miles a day and seem to feel better when I do. I take low-dose prednisone for maintenance. Am I likely to harm myself with this regimen? I am a 56 year old male.
Mazen Dimachkie, MD: Mild to moderate exercise is good for myositis. If you overdo it, your muscles will tell you as you will feel weak and achy the next day. So do not overdo it! Best wishes, MMD
Aisha Morrow, TMA: This concludes today’s discussion. Thank you, Dr. Mazen Dimachkie for spending the time today to answer questions. Thanks to all the members who participated.
Mazen Dimachkie, MD: Thank you to all of the participants. MMD