Dr. Sonye Danoff of the Johns Hopkins Myositis Center will answer questions about myositis-associated lung problems Thursday, October 6, 2011 at 12 Noon EST.
Dr. Danoff is an Assistant Professor in the Division of Pulmonary and Critical Care Medicine and Co-Director of the Hopkins Interstitial Lung Disease Clinic. She is a specialist in diseases causing fibrosis or scarring in the lung, particularly those associated with autoimmune diseases including myositis. Her research focuses on basic and translational studies of lung fibrosis. She was awarded the 2007 American Thoracic Society/Coalition for Pulmonary Fibrosis Research Award to support her research studies. She has also been a recipient of grants from the National Institutes of Health, the Mid-Atlantic American Heart Association and the American Lung Association.Ask a Question
Is there a “typical” progression to lung problems for those with a myositis?Participant:
I have been diagnosed with IBM for about seven years and thus far have not noticed any lung related diffculties. Is there a “typical” progression to lung problems for those with a myositis? I If so what kind of timeline is one looking at and generally how do they manifest themselves?Dr. Danoff:
This is a really good question and not easy to answer. Each person is really quite different so I would not say there is any “typical” progression. As I mentioned above, the early symptoms may be related to voice and cough strength or may be related to shortness of breath with activity.
Pulmonary therapy recommendation?Participant:
I have had PM for 12 years, ILD for 5 years and bronchiectasis for 4 years. PM is under control with prednisone, methotrexate, imuran and IVIG. ILD produces a frequent dry cough, bronchiectasis a productive cough. I current take a tsp of tussinex 1-2 times a day to minimize coughing. In previous on-line discussions of lung issues, pulmonary therapy has been mentioned. Do you recommend it and specifically why? What might it do to help me?Dr. Danoff:
As I mentioned earlier, I am a big believer in pulmonary rehabilitation. In addition to helping with increasing exercise endurance, most rehab programs have a great education component which can help with techniques to control symptoms of cough and shortness of breath. Another issue you that might be important is to consider airway clearance techniques to help with the phlegm from the bronchiectasis.
Dr. Danoff: I have IBM Myositis and am in a wheelchair for three years. My Doctor wanted to give me a flu shot and I refused. Should I worry about getting the Flu or Pneumonia? I do not trust vaccines with this condition. Thank you for answering.Dr. Danoff:
I understand that many people are concerned about the potential side effects of vaccines. However, there is very strong data to indicate that there is a benefit for patients with chronic medical illness (such as myopathies) from receiving yearly flu vaccine and pneumonia vaccine (pneumovax) every 5 years. I strongly recommend that my patients get vaccinated since patients with lung disease are at increased risk of severe complications from flu and pneumonia.
Increasingly out of breathParticipant:
I am a 70 yr old female diagnosed with IBM 6 yrs ago. I find I’m increasingly out of breath when walking very short distances and very slowly with the aid of my walker. I also suffer from Sjogren’s Syndrome and know that this disease can mimic symptoms of other diseases, and I would like to know if the combination of both of these diseases can precipitate this problem. My Rheumatologist checked my lungs during a normal checkup only a week ago, and he said all seemed ok. Thank you for your attention to this query.Dr. Danoff:
Hi Gail. I am sorry to hear that you are having this difficulty. There are a number of reasons that people can find themselves out of breath with activity. The lung is clearly one major reason, but the heart can also be involved. Sometimes also, because of general weakness, people find it hard to stay active and become deconditioned. I would suggest that you discuss the symptoms with your doctor. There may be additional tests or treatments that could be helpful.
When hospice doesn’t particpate, do docs have a way to help when no other solutions exist?Participant:
Hello. i have a second question for you. I should add that i am jo-1 positive along with PM and ILD. I also am keeping PCP at bay with the mepron. Am 61 years old. Never smoked, always energetic before this. I have been told that I am not a candidate for lung transplant because of the high doses of prednisone in spite of tacrolimus. Just can’t taper down to a small dose. Currently tapering to 17.5mg prednisone from last flare at 40mg per day. Generally get flareups at this level or below 20 mg. Been as high as 80 per day just a year ago. Too many risk factors. I now have a DNR. Heart is considered good. Lungs not at all. Hospice has decided they will not be able to help since I have not stopped the medications. My Rheum Doc and Pulmonary specialist have prepared me for the end. Both are excellent and well known in their fields. My days are spent doing next to nothing. Support oxygen has me more awake. Am concerned about the eventual suffocating. Have already experienced that with initial acute onset. Question: When hospice doesn’t particpate, do you docs have a way to help when no other solutions exist?Dr. Danoff:
I am really sorry to hear about your situation. I would suggest that you and your doctors go back to the hospice group to discuss how they can be a part of your ongoing care. In general, medications that maintain quality of life are acceptable for hospice care. I think that having a thoughtful discussion with your care providers is really important. Even when we, as doctors, can’t fix the problem, we are committed to maintaining our patients’ comfort and dignity. Please speak with your doctors. I feel sure that there should be a way to bring in hospice care if this is your decision.
Correlation between enlarged lymph nodes in my lungs and my dermatomyositis?Participant:
Is there a correlation between enlarged lymph nodes in my lungs and my dermatomyositis?Dr. Danoff:
The lymph nodes are structures that collect inflammatory cells from the lungs. They act as the surveillance sight for lung infections. When there is a trigger for inflammation, like a pneumonia, they can become enlarged. However, they can also become enlarged as a reaction to inflammation in the lung as can occur in dermatomyositis. One other important issue to be aware of is that the lymph nodes can become enlarged in certain forms of cancer. Because the risk of cancer is increased in dermatomyositis, it is important to be sure this possibility has been considered.
How can I keep my ILD from getting worse?Participant:
I believe ILD is not reversible, so how can it be kept from getting worse? Is it progressive? Does one always need to be on medications?Dr. Danoff:
Very important question. While lung scarring or fibrosis is thought to be irreversible, many forms of lung inflammation with ILD are reversible. So it is important to diagnose and treat lung disease early and aggressively. The decision about whether to remain on medication is still controversial. Some patients are able to come off of immunosuppressants after a period of having their lung disease quiet, others require some level of maintenance therapy to keep the lung disease under control. So this is a conversation each patient needs to have with his/her own doctor.
New treatments for interstitial lung disease?Participant:
Are there any new treatments or medications currently being used for interstitial lung disease associated with myositis or currently under investigation?Dr. Danoff:
There is a slow stream on new medications coming into clinical trial for interstitial lung disease. Unfortunately, most of these medications are being tested in other forms of ILD. However, there is a very great interest in the effect of medications being tested for control of inflammatory myositis on the rate of associated ILD. So we are getting some information from these studies.
Latest Research on ILD?Participant:
What is the latest research on ILD? Maybe targeting medications from a lung biopsy? (it was mentioned at the TMA conference by an attendee)Dr. Danoff:
Thank you for this question. There is a lot of research ongoing in myositis associated ILD. The TMA is a great source of information about it. The research is in a variety of areas including trying to understand why some patients get ILD and others don’t (genetic and environmental risk factors) as well as what actually happens in the lung during ILD (bench research on cellular and molecular changes) as well as trying to identify more effective treatments and determine which patients are most likely to benefit from specific therapy (stratification). I encourage you to discuss research trials with your doctors. Without the generous participation of our patients, we cannot hope to advance the understanding and care for patients with myositis-ILD.
How often is fibrotic lung disease associated with S-IBM?Participant:
How often is fibrotic lung disease associated with S-IBM. What % of patients and how long after diagnosisDr. Danoff:
Great question. There is a relatively low rate of fibrotic lung disease in IBM. I am not sure that I have ever seen a specific percentage in the medical literature. Apparently, this is something else we need to study.
What are my chances of developing lung disease?Participant:
I have polymyositis and also the J-1 antibody. I do not have lung disease and do have PF tests done yearly and have done well on them. What are my chances of developing lung disease? Thank you.Dr. Danoff:
Great question. We are actually doing research at present to try to answer that question. But as a rule of thumb, the longer you have myositis without lung involvement, the less likely it will develop.
Why do we get a lot of phlegm when we lie down @night and is there anything that will stop it?Dr. Danoff:
This is an incredibly common symptom among my patients. I think that there are several reasons why phlegm is worse with lying down. The first is that some secretions can pool in your sinuses (small cavities that lie in your facial bones). When you lie down at night, these puddles of phlegm pour out into your throat. A second issue can be acid reflux. This may not be associate with the classic symptoms of heartburn and indigestion. Sometimes enough acid washes back into the esophagus to trigger airway inflammation. I generally ask patients to consider both possibilities. Reflux is improved by avoiding eating for 4 hours before lying down as well as avoiding high fat foods in the evening (chocolates and ice cream especially). The head of your bed can be raise 4-6 inches using blocks under the foot of the bed. Other approaches include using medications to block acid production in the stomach. For the sinus involvement, many patients find sinus rinses are helpful or alternatively nasal steroids to decrease the inflammation in the sinuses.
IVIG and ILDParticipant:
I was diagnosed with Polymyositis in 2002, I have been receiving IVIG since the diagnosis. I was not aware of the possibility of developing ILD until recently when I read an article about it.I am not sure I have been monitored for development of ILD and recently have developed this frequent cough. I was diagnosed with Asthma in 1995 or 96. Should I be concerned?Dr. Danoff:
Hi Marilyn. I think that if you are concerned about the cough being a sign of interstitial lung disease, it would be important to discuss this with your doctor. You have pointed out very well that breathing symptoms are not specific indicators of ILD. In other words, the cough might be due to asthma. But it is relatively simple to determine the cause with a careful exam, history, pulmonary function testing and a chest CT if this is indicated.
Is it possible to have Lung Disease or Lung Cancer and it not show up in an X-Ray?Participant:
Doctor: Is it possible or probable to have Lung Disease or Lung Cancer and it not show up in an X-Ray? Please explain if answer is yes.Dr. Danoff:
Hi Bill. Chest xrays are a great screening method to look for lung disease in someone who has lung symptoms (cough, phlegm, shortness of breath). A chest xray may be sufficient to make a diagnosis. However sometimes it is necessary to use a chest CT scan to get better resolution of more subtle changes within the lung as well as to see areas of the lung that can be more difficult to evaluate with a chest xray such as the very bottom of the lung.
Correlation between Interstitial Myositis and Interstitial Lung Disease?Participant:
Is there any correlation between Interstitial Myositis and Interstitial Lung Disease?Dr. Danoff:
There are a number of forms of inflammatory myopathy (polymyositis and dermatomyositis) that can be associated with interstitial lung disease. In these cases, the lung is a target of injury by the immune system (causing the ILD) in the same way the muscle is a target (causing the myositis) of the skin is a target (causing the skin rash in DM). Interestingly, however, in some people, the disease is dominated by one organ—either the muscle or the lung or the skin in DM. So the activity of disease in one organ doesn’t always reflect the activity in another organ. In other words, some people can have almost completely muscle disease, while others have almost completely lung disease.
Because I have IBM is it a given I’ll have lung problems?Participant:
Because I have IBM is it a given I’ll have lung problems? I’m a 72 female who was diagnosed this spring.Dr. Danoff:
Inclusion body myositis (IBM) can be associated with lung issues, but generally not interstitial lung disease. More commonly, the lung is affected as a result of the muscles that support breathing becoming weak. This is certainly not an issue for all patients with IBM. It is helpful to discuss any symptoms of shortness of breath with your treating doctor, so if there is a concern, appropriate tests can be ordered.
Is there a direct correlation with the Methotrexate and my lung changes?Participant:
Hi Dr. Danoff, thank you for taking my question. I’ve was diagnosed with ILD three years ago and PM one year ago. Recently my Rheumatologist put me on Methotrexate at .6 which I took weekly SQ for 3 months. My Pulmonologist was very much against this. I noticed slight lung changes such as chest tightness, sob with exercision, cough with clear sputum. Is there a direct correlation with the Methotrexate and my lung changes? I have since stopped taking it but struggle with the same symptoms. Currently I am taking the following: Cytoxin 750mg IV/monthly, Prednisone 30mg/day.Dr. Danoff:
You have asked a question that rheumatologists and pulmonologists wrestle with frequently. Is a symptom due to the disease (PM and ILD) or the treatment for the disease (for example, methotrexate (MTX), but it could be another medication)? There is no simple answer. In general, if we think that a symptom, like chest tightness or shortness of breath with activity might be due to a medication, we stop the medication. If the symptom goes away, then it makes us think that it may have been due to the medication. If it doesn’t go away, then it makes us think that it was more likely due to the disease. At present there isn’t a cleaner way of answering this question.
Pulmonary Hypertension and DermatomyositisParticipant:
Hello My name is Sue and I was diagnosed 14 years ago with Dermatomyositis. Recently, I was diagnosed with pulmonary hypertension and diastolic dysfunction. I was hoping you could expand on the treatment and outlook of this situation. Thank youDr. Danoff:
Hi, Sue. Pulmonary hypertension (PH) occurs when the pressure in the right side of the heart (the side which pumps blood to the lungs) gets too high. This can occur for a number of reasons [including medication use, weakness in the left side of the heart (left heart failure), blood clots to the lung (pulmonary emboli), sleep apnea], but sometimes it occurs as a part of an autoimmune disease. The important first step is to work with a specialist to determine what caused the PH, because the treatment is linked to the cause.
Is scarring of the lung part of IBM?Dr. Danoff:
Interesting question. Unlike other forms of inflammatory myopathy, inclusion body myosits (IBM) is generally not associated with scarring of the lung (also called fibrosis). There can be other issues with the lung related to muscle weakness, but scarring is far less common than with dermatomyositis or polymyositis. That said, there are rare occasions when patients with IBM can have lung fibrosis.
Collapsed lunch a frequent issue?Participant:
I am currently suffering from my second collapsed lung in less than 6 months. I am curious to know if during your studies of myositis you have found this to be a frequent issue?Dr. Danoff:
I am sorry to hear about the collapsed lungs (pneumothorax). There are actually a number of reports linking dermatomyositis to pneumomediastinum (spontaneously developing air in the middle of the chest around the heart, great vessels and large airways). When a pneumomediastinum occurs, there can also be a lung collapse (pneumthorax). To the best of my knowledge, there is no clear explanation as to why these seemingly separate issues (DM and pneumomediastinum) occur together.
Does the course and/or treatment of dermatomyositis/ILD run differently based on its origin?Participant:
Does the course and/or treatment of dermatomyositis/ILD run differently based on its origin (ie: drug induced/environmental/viral/unknown)?Dr. Danoff:
This is a really interesting question and one which we and others are trying to understand. There is currently a multi-center study run by the NIH (headed by Dr. Fred Miller) recruiting, called MyoRISK, which is aimed at identifying potential environmental triggers for myositis and interstitial lung disease. I hope that this study and other which are ongoing will help to provide better answers for your question.
Can lung capacity loss can be regained?Participant:
I have had polymyositis with ILD for three years. While the disease is being managed, I have lost about 20% lung capacity. Is there any way this loss can be regained?Dr. Danoff:
This is an interesting question. There are a number of reasons why lung function (particularly lung volume as measured by forced vital capacity and total lung capacity) can decline. The first reason is related to inflammation and scarring in the lung. Inflammation is generally thought to be reversible, while scarring or fibrosis is believed to be permanent.
However in myositis associated disease there is also the contribution of muscle weakness. Specifically if the diaphragm (the large muscle that separates the chest from the abdomen) becomes weakened, then the lung volume can decline without the lungs per se being involved. As muscle function improves, then lung volume usually increases. A second issue with the muscle weakness is that if one’s weight increases due to medications or decreased activity, there can be an apparent decline in lung volume from the pressure up on the diaphragm.
I am a strong proponent of the benefits of pulmonary rehabilitation. These are monitored exercise programs designed for people with lung disease. They are generally about 12 weeks long and meet 2-3 times per week. The goal is to provide a safe environment to achieve increased exercise tolerance. I find these programs are very effective both for increasing endurance and helping to control weight. Your might consider discussing this with your doctor if you haven’t done so already.
Do you think Pulmonologist be more agressive in their testing for ILD in Patients with Classical Dermatomyositis and overlap of Sjogren’s, Raynaud’s Phenonema, Sleep Apnea and GERD who have been on treatment using MTX Ingections (1CC) since diagnosis, Prednisone (Max Dose for three weeks), Arthrotec, IVIG Therapy, Ambien, Celexa, Exovac, 50,000 IU of Vitamin D for two past years, and Celebrum Ultra Multivitamins for past four years with no remission since diagnosis. If so what testing should be done and how often?Dr. Danoff:
I think that the ideal situation for every patient with autoimmune associated interstitial lung disease is to have a pulmonologist and rheumatologist who work together closely. This is the model we use at Hopkins and I think it is increasingly being adopted at other centers as well. In general, the testing for lung disease includes a thorough history and physical exam. Pulmonary function testing is helpful for monitoring lung disease. We generally recommend that these be done 3-4 times yearly, although more often may be appropriate for someone just starting treatment and less frequently might be appropriate for a patient with relatively stable lung disease. In addition a high-resolution chest CT scan can be useful early in the process of diagnosis and may also help if there is a change in symptoms. Sometimes a lung biopsy is needed to provide additional information for making a diagnosis. In addition to these diagnostic studies, we recommend that a walking (ambulatory) oxygen saturation test be done periodically to assess for the need for supplemental oxygen. Sometimes this is done as a part of a six-minute walk test. These are issues that are important to discuss with your doctor.
Advice for someone with lung disease?Participant:
Hello Dr. Danoff, I am a 55 year old woman whose lung disease began after a pulmonary embolism in 1997 and has “gradual declined” (thank God for me) over the years to about 47% lung volumes to date. My lung biopsy in 2004 showed severe fibrosis in my lower lungs and middle right lung and I was given 6 months to live back then? Very grateful they were wrong! Recently my doctors, I believe, have finally pinpointed my disease as Antisythetase Syndrome with NSIP as I have a positive Antijo 1 after all these years of ever changing diagnoses. I’m wondering if the pulmonary embolism or the Heparin I was put on could have triggered the autoimmune disease and lung disease? A month after the embolism I had SEVERE joint pain and fatigue and was diagnosed then with R/A. The joint pain has now lessened and has been replaced with muscle pain, weakness and fatigue. I am back on a low dose( 3 mg daily) Warfarin now because I recently got a port to receive my IVIG infusions every 4 weeks. Could there be a link to the heparin/Warfarin with my autoimmune and lung disease? Nervous about taking Warfarin now. Thank you Dr. Danoff for taking my question. Are you excepting referrals or trial studies as I feel it would be good idea to make an appointment with you for a 2nd opinion. I am currently being treated at Mass General in Boston MA at this time for Pulmonology and Rheumatology and I love my doctors but neither specialize in my specific type of lung disease. I appreciate any advice you may have to share.Dr. Danoff:
Hi Lin. Thank you for sharing your story. In my experience, blood clots to the lung (pulmonary emboli) are more common in patients with autoimmune interstitial lung disease than in patients with other forms of interstitial lung disease. So I suspect that the blood clots might be a part of the autoimmune disease spectrum. Of course there are many other common reasons for the development of blood clots (pregnancy, leg trauma, inactivity, genetic factors). Hopefully research at Johns Hopkins and other centers will help to answer this question.
What are the early symptoms of lung disease associated with IBM?Participant:
I was diagnoised in 2002 with IBM thus far I have not had any serious lung problems. What are the early symptoms of lung disease associated with IBM?Dr. Danoff:
The symptoms of lung involvement in IBM generally reflect the decreased strength of the muscles of breathing and may include change in voice (getting quieter), change in cough (getting weaker) or a decrease in exercise level due to shortness of breath. The lung function can be checked by your doctor by doing pulmonary function testing to determine the forced vital capacity (FVC).
I have IBM. My lung capacity has been slowly and steadily decreasing and is now down to 1.1 liter.I have no signs of any lung disease, just a weakening diaphragm. My pulmonologist wants to start me on supplemental oxygen and says at some point I’ll have to decide if I want to go on a ventilator. Is this common for IBM?Dr. Danoff:
Inclusion body myositis can affect the muscles of breathing (particularly the diaphragm). When this occurs, the lung capacity may decline over time. The use of oxygen is generally dependent on whether the blood oxygen level drops either at rest or with activity. This is tested by a pulse oximeter device that is placed on the finger. The pulse ox will determine what percent of the blood is saturated with oxygen. If this number is below 88%, we generally recommend the use of supplemental oxygen.
The other major function of the lung (in addition to absorbing oxygen) is to rid the body of the waste gas, carbon dioxide (CO2). If the lungs are no longer able to remove this gas adequately, the level rises in the blood and can have serious consequences. The ability of the lung to remove CO2 can be supplemented by a form of mechanical ventilation. There are several forms of mechanical ventilation which can be used for this purpose. It is important to discuss these options with your doctor long before any decision needs to be made, so you have the opportunity to decide what course of action is right for you.
My PM is considered in remission, drug induced but stable now for 3 years, my question is the ILD, I am told that is also stable and my last PFT test was comparable to the previous one 6 months ago, so now I do not have to have them but once a year. My Pulmonologist feels that as long as the PM is stable and I am just on a maintenance dose of Imuran 75mg. that I probably will not have any flairs from the ILD or the PM. Do you agree with this plan? The rheumatologist is considering lowering the Imuran to 50mg in another 3 months. My cpk is now at 94 They said all the inflammation in the lungs is gone and no rails are heard at all. Thanks, faithndallas/PM/ILDDr. Danoff:
Thank you for your question. I am afraid that I can’t comment on your specific situation since I don’t know all of the details. In general terms, some patients with polymyositis (PM) and interstitial lung disease (ILD) do reach a “quiet” state. The disease hasn’t gone away, but it is much less active. When that occurs, it is sometimes possible to lower the amount of immunosuppressant medication (like Imuran) to a maintenance level. It is important to continue to have follow up for both the ILD and the PM, because these diseases can reactivate months or even years later. We also know that some patients with PM and ILD have well controlled muscle disease, but their lung disease remains active (usually with symptoms of cough and shortness of breath). So we recommend routine follow up with pulmonary function tests (PFTs) to measure the lung function and clinic visits to discuss symptoms and check the lung exam even when the disease is quiet.
We are at the end of another great discussion! Thanks to all the members who participated today. A special thanks to Dr. Danoff again for being with us and graciously spending the time to answer your questions.Dr. Danoff:
Thank you for your questions. I wish that I had time to answer all of them. But you have really pointed out how important lung disease is in myositis and how critical it is for us to move forward with research to find the cause and the cure. Thanks for allowing me to be involved in this discussion. Sonye Danoff, MD, PhD