Myositis and Lung Disease

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.

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.

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.

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.

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.

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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

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.