on March 09, 2015
We are pleased to welcome Dr. Dana Ascherman today to answer questions about myositis and lung disease. Dr. Ascherman is a rheumatologist and an associate professor with the University of Miami Health System. He has a special interest in the overlap between myositis and lung disease, and co-founded the Autoimmune Interstitial Lung Disease Clinic in Miami. He is also a member of TMA’s medical advisory board and board of directors. Dr. Ascherman has already received a number of questions and will select those that are of the greatest general interest. Although your name will appear with your question during the course of the discussion, it will not appear on the final transcript. Thanks so much for being with us today, Dr. Ascherman.
Dr. Ascherman: Thank you. I’m pleased to be “here.”
TMA Member: I am a 68 year old femaile who was diagnosed with polymyositis with interstitial lung disease with the anti-jo antibody spring 2010 (anti synthetase syndrome.) I have been treated with two pulse infusions of solumedral in 2010 and 2012 followed up by prednisone at 80 mg, eventually tapered to 5 mg currently and replaced by mycophenolate (cellcept) starting at 3000 mg and now at 2500 mg. I have not had a relapse since May 2012. I know two of the side effects of taking mycophenolate and prednisone are being immuno suppressed and being susceptible to certain cancers.
1) What level of mycophenolate should one strive for to still keep the ILD under control and to better be able to fend off lung infections or cancers ?
To monitor the effects of cellcept and the progress of polymyositis with ILD:
2) How often should one have blood lab tests and which tests?
3) How often have CT of chest? How often have chest x-rays?
4) How often have pulmonary function tests ?
5) What is the long term probability of infections while on cellcept?
6) What is the long term probability of cancers (which ones) while on cellcept?
7) What side effects become more pronounced after 3+ years on cellcept? My hair seems to be thinning, will that continue?
8) As long as cellcept is working and I am stable, I will stick with it. However, if needed, what other medications seem to be working for ILD with polymyositis?
Thank you for your time and expertise.
Dr. Ascherman: 1. We don’t know, but probably 1000 mg bid 2. 6-8 weeks 3.Depends on symptoms, PFTs 4. 4-6 months 5. Unknown, but depends on multiple factors, including WBC count, prophylactic immunizations, exposures 6. Unknown, but not significant 7. Will probably continue, but most side effects occur in initial treatment period 8. tacrolimus
TMA Member: Good day Dr. Please could you elaborate a bit on the risk of lung cancer with polymyositis. Is there an increased risk for those who have not had any lung disease from the myositis? How long does the risk persist (does the risk persist beyond 15 years in those who are in complete remission)?
Dr. Ascherman: Remarkably little data—more with DM than PM. May be increased risk associated with long term interstitial lung disease, but few natural history studies to adequately answer this question. Overall, risk pales in comparison to risk associated with smoking.
TMA Member: Dr. Ascherman, I was diagnosed with Myositis IBM in 2013. Prior to that, In 2010 I was diagnosed with the uncommon Castleman’s disease and a non malignant mass was removed from my upper right lung quadrant. My Castleman’s medical specialist and Neurologist said there was no connection. I still wonder. Is there is a clinical trial or treatment for this dual immune system dysfunction?
Dr. Ascherman: No clinical trial for this combination. Also, no clear association given that the primary process in IBM may not be immunologically-mediated.
TMA Member: If exercise helps our trunk, arm and leg muscles damaged by myositis, is there ANY exercise we can do to help our damaged lungs? I know ILD scars lung tissue but for the lung tissue still functioning, can I do something to make it more effective?
Dr. Ascherman: Pulmonary rehabilitation may be quite helpful.
TMA Member: Is ILD an autoimmune disease? How about bronchiectasis? Is this a case of overlap syndrome?
Dr. Ascherman: ILD is thought to represent autoimmune attack of lungs, paralleling inappropriate targeting of other tissues such as the muscles. The link with autoantigens/autoantibodies such as Jo-1 supports this concept. We consider this part of disease, rather than overlap syndrome—but there are certainly overlap syndromes (e.g., MCTD) with some features of myositis and ILD vs. other features of SLE, Sjogren’s, RA, and/or scleroderma.
TMA Member: If my ILD becomes bad enough for the doctors to consider a lung transplant, how do they decide to replace one or both lungs? And after the surgery, wouldn’t the ILD just start all over again?
Dr. Ascherman: Decision of single vs. double lung transplant is controversial—involves balance between short term survival (may be better for single lung transplant) and long term survival (may be better for double lung transplant), organ availability. The issue of recurrence is a general one to be considered in organ transplantation for autoimmune disease; figures not well established, but the disease does not immediately re-develop—perhaps altered immunologic setpoints.
TMA Member: Your thoughts on Rituxin for ILD. Is it being offered earlier than waiting until the immunosuppressants are not working? I know of a few people on it and it seems to up help. Thank you.
Dr. Ascherman: This would be my biologic agent of choice if no response, or contra-indication, to chemotherapeutic agents such as Cellcept, Imuran, or tacrolimus—but only anecdotal literature to support efficacy of Rituximab.
TMA Member: I was diagnosed with DM in Aug2014 then hospitalized and diagnosed with Interstitial Lung Disease in Nov2014. When I was hospitalized for my extreme shortness of breath in Nov they put me on high dose steroids and almost all of the ground glass opacity disappeared but I still have a nodule in my lungs. Is this typical? They were going to do a lung biopsy before the steroids improved everything. If you have DM or PM with lung involvement should you get a biopsy to ensure early detection of something serious?
Dr. Ascherman: Nodules can definitely occur in PM/DM, and Dr. Lisa Christopher-Stine (Johns Hopkins) has been studying this issue. The bottom line is that the nodule should be followed with serial CT scans—if change in size or morphology, the nodule should be biopsied.
TMA Member: I was diagnosed finally in April of ’14 and had pulmonary testing as I was always short of breath. My testing was slightly worse than the time before that which was 6 months prior but it was still normal. I get very short of breath when I’m having a bad muscle day and when I stand. My question is when you are short of breath due to muscle exhaustion does that have anything to do with lung involvement? I am scared to death of my lungs being affected and want to be alert to signs of such. I understand you are not my doctor and I will talk to my DM dr about this but would love your input. Thank you so much for your time… 🙂
Dr. Ascherman: Shortness of breath in myositis can reflect multiple factors, some extrinsic to lung tissue (muscle weakness/hypoventilation, aspiration, infection) and others intrinsic (ILD=Interstitial Lung Disease). Combination of imaging and PFTs (Pulmonary Function Tests–including Maximum Inspiratory and Expiratory Pressures) should be able to address relative contributions from muscle weakness and/or ILD.
TMA Member: If my stats have stayed the same and are ok, I am wondering why I am experiencing being more short of breath. My DLCO is at 65% and my ck at 73. My pulmologist mentioned it may be deconditioning of the lungs. Does this mean I need to do walking, etc? Thank you.
Dr. Ascherman: Could be cardiovascular deconditioning or “hypoventilation” due to previous muscle damage. Cardiology evaluation and pulmonary rehabilitation are both important considerations–the latter can be helpful in terms of providing guidance for physical activity.
TMA Member: Dr. Ascherman, I am a 74 year old female. In Feb, 2008 I was diagnosed with PM and positive for Jo-1 but have no symptoms of an ILD. Is it still possible for me to develop an ILD? While I do not have an ILD, my mild/moderate asthma that I have had throughout my adult life became “severe” 3 years ago. Is it possible that Jo-1 could have affected the asthma? Thank you for taking your time to answer our questions.
Dr. Ascherman: ILD can be “subclinical”—so important to have full battery of PFTs, imaging to rule out this potential disease complication. Typically, airways are not directly involved in this process—but inflammation surrounding airways could trigger increased (hyper)reactivity of airways.
TMA Member: Diagnosed with ADM in february 2014, diagnosed with cryptogenic organising pneumonia june 2013 post open lung biopsy. initially treated with prednisone and methylprednisolone 1000mg IV. required nissen funduplication 28 august 2013 as diagnosed with silent aspiration as well, some bronchiectasis on left base. TLC 50% and DCLO 31% saturation 80% ORA at rest, 72 on minimal exertion. HR 145 ANA 2700, Myositys panel: SSA-Ro52 +ve, Ku +ve, CRP normal levels Home O2 24/7 Tx escalated with 6 months of cyclophosphamide IV. with good effect TLC remained 50% DLCO improved to 41%. saturation ORA 97%. 89-90% on minimal exertion. HR improved 75-80 at rest. 110 walking. currently on umiran 150grm and prednisone7mg. x- tolerance improved from 10mts (june2013) to currently able to walk 8,000 steps per day My question is how do you determine when you can stop the medications. so far the only indication for me has been the LFT. my ANA has improved to 640. is any blood test that may hep to assist when you increase or decrease medications for the lungs. I really would like to decrease my medications as the prednisone has induced diabetes and the imuran has started to affect my liver function. at the same time i would not like my lungs to be affected.
Dr. Ascherman: No great blood markers to follow activity of lung disease at present. Currently, we rely on a combination of clinical symptoms, PFT changes, and imaging abnormalities to gauge disease activity vs. stability—which influences treatment decisions and duration of therapy.
TMA Member: What type of new treatments are available for people with ILD involved with myositis? what is your opinion of stem cells transplant? how would it work?
Dr. Ascherman: No real “new” treatments are available for myositis-associated ILD, but we are gathering more information about response to existing chemotherapeutic agents and biologics. Perfenidone and other tyrosine kinase inhibitors have garnered some attention in IPF. Stem cell transplant (Mesenchymal Stem Cells) being looked at in IPF, but not formally studied in myositis-associated ILD. Part of difficulty is that we don’t know if effects of Mesenchymal Stem Cells are anti-inflammatory, regenerative, or both.
TMA Member: I have rhematoid arthritis,pulmonary fibrosis,polymositis,type too diabetes,i was wondering what type of medication i should be taken,considering i am English and do not have to pay for the medication,so the cost is not a great factor.
Dr. Ascherman: Medication choice depends more on subtype of ILD than disease association (RA vs. myositis), though distribution of ILD variants may be different in these diseases. We are using agents such as Cellcept and tacrolimus in myositis (as well as RA)-associated ILD, but sometimes use very powerful agents such as cytoxan/cyclophosphamide.–all of which are available in the UK.
TMA Member: Hello, I have h-IBM and silent asperation. During the last year I have had pneumonia and 3 chest infections. Just last week I finished the latest anti biotics. I now get so out of breath it can be frightening especially when I first get up. My diapram is weak so I cannot cough effectively. My lungs are now clear. I have now lost 2 st ( 28lb ) since last may for which I blame my loss of appetite due to feeling unwell and pureed meals. I cannot exercise to get my self in better condition due to my IBM. I live in the UK and am 74 yrs of age. Diagnosed 6 years ago. Weight now 8st 6lb ( 118 lbs )
Have you any suggestions as to how I can improve my lifestyle?
Dr. Ascherman: Nutritional counseling to ensure adequate caloric, protein intake is very important. Working directly with Physical Therapy and even pulmonary rehabilitation would also be useful.
TMA Member: What tests are performed to check for lung disease associated with myositis?
Dr. Ascherman: Usually, we look at a combination of the following: 1. Clinical abnormalities (“rales” on examination) 2. Autoantibodies 3. PFTs 4. Imaging/HRCT.
TMA Member: I have been diagnosed in 2013 with (dermatomyositis) anti synthetase syndrome, anti-jo 1 and ILD. (I also have Renaud’s, vasculitis with minor necrosis at times, gottrons papule) i have a couple of questions 1- With the quick description of my health would you call this dermatomyositis? or because of the antisynthetase syndrome you would consider this another type of myositis? if so how would you name it? 2- If i had lesions in my lungs in 2006 but was not investigated further, could this mean it started at that time? 3- Approx how many patients with a similar dx as mine have you treated? 4- I know about the poor prognosis, what is the average in years pt lived with this dx?
Dr. Ascherman: 1. Yes (dermatomyositis is the correct diagnosis with Gottron’s papules); unifying factor is Jo-1/anti-synthetase syndrome, rather than DM vs. PM. 2. Could have started even earlier, as these lesions may have been developing asymptomatically. 3. Completely depends on subtype, severity, damage associated with ILD—5 year survival rates in general are lower with myositis-ILD, but still >70% 5 year survival.
TMA Member: Living far from any myositis specialist, is there a way i could communicate with a physician where i could ask specific questions about my diagnosis and treatment? Or how could i arrange something similar?
Dr. Ascherman: You can try corresponding with TMA Medical Adviosry Board member or other myositis expert, though it’s impossible for any physician to remotely perform a truly adequate assessment.
TMA Member: What are the signs of lung disease and myositis? Thank you.
Dr. Ascherman: Lung disease in myositis ranges from asymptomatic to life threatening dyspnea (shortness of breath). Non-productive cough, exertional dyspnea are early symptoms, but not specific for ILD. We always need to worry about hypoventilation (due to muscle weakness), aspiration, infection, etc.
TMA Member: Good day Dr. Ascherman, first of all thanks for your time. I was diagnosed with polymyositis approximately ten years ago after having the mumps (aged 21). Treated with steroids as standard and all cleared up within a few months. During the PM episode no lung involvement was picked up by the Doctors. Have been in complete remission ever since with no flare ups. Can myositis ever cause a refractory wheeze that occurs when exhaling? Respiratory Doctors have ruled out asthma, emphysema and ILD (have had full range of pft’s (incl gas transfer) and chest ct which all came back negative/within normal range). However, I have a wheeze when exhaling, that is refractory and non responsive to steroid inhalers. Could it be myositis related or more likley something completely unrelated?
Dr. Ascherman: Likely unrelated—need to look at flow volume loops for variable intra/extra-thoracic airway obstruction (technical aspects of lung mechanics)—but hopefully your pulmonologist has already performed these studies.
TMA Member: Dr. My name is Mike Harper. I am a 56 and was diagnosed with IBM in 2006. Since then I was diagnosed with atelectisis of the right lung. This was following multiple upper resp infections. Is this common for someone who has been on high dose prednisone for an extended time. What happens to the lung? Does the body rid itself of it or does it get calcified as other things in the body sometimes do?
Dr. Ascherman: Atelectasis unlikely related to prednisone use. This process usually results from hypo-inflation, weakness of respiratory muscles rather than intrinsic lung disease (though could be sequela of lower respiratory tract infection). May be some tissue remodeling over time, but should not become calcified.
TMA Member: I was diagnosed with ASS, DM and ILD in June 2011, when I turned 50. I spent nine months in hospital with one month of that in ICU. I had quite a few complications with DVT in both legs, allergic reaction to Dapsone, Nocardia infection for 18 months and now have severe Osteoporios having had around 6 fractures but with help of my wonderful Immunologist in Brisbane I survived and have been in medical induced remission for approximately 18 months. My question is does my ILD cause my increased inability to handle heat/humidity or is it the DM? I live in a very humid city and suffer a lot with maintaining the ability to function in the heat. I returned to work in 2014 for 3 days a week as a teacher but I get very tired and overheat extremely easily and become very breathless when I overheat. I perspire profusely and my face reddens. My lung capacity at my last LFT was 67%. Will this pressure of overheating exacerbate my lung condition and in turn my life expectancy?
Dr. Ascherman: Both the ILD and DM may make it more difficult for you to handle physiological stressors, including heat/humidity—though the mechanism is not immediately clear for heat/humidity. As long as you don’t become dehydrated, which increases cardiovascular stress, heat/humidity in and of itself should not lower life expectancy. P.S. I do not like this acronym for the anti-synthetase syndrome, despite its fairly widespread usage in the literature.
TMA Member: What is your recommendation regarding devices I can use at home to help with my breathing/coughing? Flutter? Vest? Spirometer?
Dr. Ascherman: All could be helpful, depending on level of ILD vs. bronchiectasis. However, I would defer to the pulmonologist, in this case.
TMA Member: Diagnosed with idiopathic BOOP (COP) in January 2006 based on lung biopsy. No symptoms associated with polymyositis, therefore, no autoimmunity tests conducted at the time. Treated with high-dose prednisone, tapered over one year. Lung disease stable since that time. Also have elevated right hemidiaghragm with paralysis. Placed on statins in late 2007, developed overt symptoms of PM (little or no DM). Diagnosis of polymyositis based on muscle biopsy and autoimmune assays, positive for anti Jo-1. Treated with prednisone since 2008 with Cellcept since 2011, CPK generally normal or slightly elevated. In November 2012, after exposure to a virulent illness, I suddenly developed marked O2 desaturation on exercise with recovery within 2-4 minutes on cessation, plus periodic “flare ups” of a bronchitic nature. I finally forced the issue with my pulmonologist, who continues to exhibit little curiosity. I am currently undergoing specialized testing at a specialized diagnostic clinic, am using O2 at night. Cardiac tests are grossly normal. I do not want just the symptoms treated; I would like a diagnosis – WHY is this happening, and why so suddenly a couple of years ago? Is there any diseaseprocess that should be considered that may be rare or unusual given this scenario?
Dr. Ascherman: Do you have currently have radiographic evidence of ILD? Flare ups can be abrupt (Acute Exacerbations, also seen in Idiopathic Pulmonary Fibrosis), may be triggered by additional immunologic insult/challenge such as infection.
TMA Member: What are your thoughts on a maintenance dose of 6.5 mg of prednisone for ILD? I have Antisynthese syndrome and have been on prednisone for 4-1/2 years along with Cellcet (3,000) mg. I have differing opinions from two rheumatologist’s – one says to stay on prednisone and one says to come off prednisone. I have 78% TLC and 65% DLCO. Thank you.
Dr. Ascherman: Goal should always be lowest dose of prednisone—can be facilitated by so-called steroid-sparing agents such as Cellcept/MMF or tacrolimus.
TMA Member: Lung disease involvement in myositis many times are treated by bronchodilators and relievers. I responded only 3% to bronchodilators during the LFT so i stopped using Ventoline. i have been prescribed seretide puffers.
My question if our problem are deep down in the tissues why do we need to use relievers puffers?. some people need it as they had asthma prior their lung involvement with Myositis. or people with ILD still get the benefit from the relievers?
Dr. Ascherman: Bronchodilator therapy does not have a direct role in the treatment of ILD, which affects lung tissues primarily and only secondarily involves airways. Some inhaled therapy can promote mobilization of secretions that can be useful if bronchiectasis—but not well established.
TMA Member: Diagnosed in 2012 with sIBM. Have recently been experiencing a lot of sputum. Had 2 x-rays within last 4 months and they came back OK. Is this a normal part of what is expected or is there anything I can do to help with this? (have tried antibiotics, mucinex and not much difference).
Dr. Ascherman: Issue is cause of sputum—post-nasal drainage, reflux, aspiration, etc. Ascertaining cause would lead to more targeted therapy.
TMA Member: What tests should be conducted to ascertain whether one has lung disease related to myositis? Thank you.
Dr. Ascherman: Please see answer to earlier question.
TMA Member: As a patient with DM, I am wondering at what point I advocate for testing for lung involvement. Are there certain markers that indicate a higher likelihood of developing this (i.e., testing positive for certain antibodies, etc.)?
Dr. Ascherman: Screening for lung disease is always prudent, though the diagnostic study of choice is not well established. I usually screen with PFTs and then make a decision about imaging. Autoantibody assessment can be helpful prognostically, since patients with certain antibodies (e.g., Jo-1) are much more likely to develop ILD.
TMA Member: Dr. Fred Miller was conducting a study aimed at identifying potential environmental triggers for myositis and ILD. What has been learned from this study? Thank you.
Dr. Ascherman: This study is ongoing, and Dr. Miller has not conveyed any results from interim analyses.
TMA Member: In my mid-40’s, I was diagnosed with COPD after experiencing shortness of breath upon any exertion. My pulmonologist seemed dumbfounded though, because I had neither ever smoked nor knowingly been exposed to other antagonists. In my early-50’s, leg weakness, difficulty in climbing stairs, and falls led to a biopsy and subsequent diagnosis of IBM. Is it common for lung problems to be the precursor to IBM?
Dr. Ascherman: IBM is not commonly associated with “intrinsic” lung disease such as ILD–but can certainly be complicated by aspiration pneumonia/pneumonitis.
TMA Member: Is lung disease common with IBM patients?
Dr. Ascherman: See answer to previous question–lung disease is not common as a primary process in IBM, but can develop as a secondary complication of processes such as aspiration.
TMA Member: I have bronchiectisis, CVID, polymyositis, connective tissue disease and diffuse lymphocytic infiltration of multiple organs (B cell). I’ve lost pituitary & pancreatic function and have bone lesions. Have you any iinsights regarding this disease process and how to address treatment? I’ve had more lung infections and have shortness of breath this year. Polymyositis is resistant to the treatments (20 mg. prednisone, 400 mg plaquenil). Tried 3 10 mg. doses of methotrexate and got thrush. Would appreciate any treatment suggestions. Thank you.
Dr. Ascherman: This is obviously an unusual and difficult combination. Because of CVID and risk of infection, your doctors have undoubtedly been reluctant to use potent immune suppressing medications. Have you been on IVIG (not just replacement doses, but therapeutic doses)?
TMA Member: I have noted a relatively long lasting positive affect(perhaps 6 months) on my interstial lung diesease after taking rituximab (100mg)(two doses). Should I take some dose periodically as a maintenance measure?
Dr. Ascherman: There is no formulaic answer to your question–depends on your clinical situation/symptoms, PFT parameters, and imaging studies. The bottom line is that re-treatment should not be “scheduled” every 6 months without specific indications for re-treatment.
TMA Member: I have been diagnosed with DM and connected tissue disorder lung disease. My treatment is Prednisolne and Azathioprine – is this the right choice for my condition or are there better treatment options?
Dr. Ascherman: Unfortunately, we don’t have clinical trials to prove superiority of one agent over another for myositis-associated ILD. If Imuran is effective, stick with it; othewise, options include Cellcept, tacrolimus, cytoxan, or perhaps Rituximab.
TMA Member: Does the level of autoantibodies indicate whether someone will get lung disease? I have amyopathic dm and I know my chances of getting ILD are very high, but I have had ADM for 17 years and so far do not have ILD.
Dr. Ascherman: Absolute levels of an autoantibody probably not as critical as the presence vs. absence of that antibody for predictive purposes. If you haven’t had ILD after 17 years, the likelihood is that you won’t experience this problem (which is good).
Aisha Morrow, TMA: This concludes today’s discussion. TMA would like to extend a special thank you to Dr. Dana Ascherman for spending the time to answer your questions. Thanks to all the members who participated.
Dr. Ascherman: I apologize for not answering all of your questions, but I hope that this was helpful for all.