Online Health Chat with Brian Southern, MD
Tuesday, September 20, 2016
Interstitial lung disease (ILD) is a group of conditions that cause scarring of the lungs. ILDs include forms of pulmonary fibrosis and interstitial pneumonia, as well as ILDs associated with connective tissue diseases, tobacco use and exposure to environmental and occupational toxins.
Since ILD is not a single disease but a group of more than 200 different pulmonary disorders, it can be confusing to understand exactly what you are dealing with, how a diagnosis is made and who needs to be involved.
Although scarring is mostly irreversible, medications can help. Relief of symptoms can be achieved through pulmonary treatments and oxygen therapy. In select cases, surgery and lung transplants may be options. The goals of therapy are to preserve current lung function and slow disease progression.
About the Speaker
Brian Southern, MD, is a staff physician in the Respiratory Institute and assistant professor of molecular medicine at the Cleveland Clinic Lerner College of Medicine of Case Western Reserve University. He has a secondary appointment as an associate staff scientist in the Lerner Research Institute Department of Pathobiology, where his research focus is idiopathic pulmonary fibrosis (IPF).
Dr. Southern explores how fibroblasts and myofibroblasts (the scar-producing cells in IPF) interact with the lung tissue matrix to propagate fibrosis. He has identified that the molecular motor protein, non-muscle myosin II, is a critical molecule driving fibrosis, and is further characterizing the myosin II pathway in fibroblasts in order to identify novel targets to halt fibrosis in patients with IPF.
In addition, Dr. Southern conducts an outpatient clinic where he cares for patients with interstitial lung disease and sarcoidosis. He serves as a co-investigator on a number of clinical trials for patients with IPF. He also supervises trainees in the Pulmonary Fellow Interstitial Lung Disease Clinic and on the inpatient pulmonary consult service.
Let’s Chat About Interstitial Lung Disease
aslycy: I had polio at age 5, which was treated at that time with the Sister Kenny procedure in the hospital for one month. I've had scar tissue on my left lung since and shortness of breath. Now, at age 80, breathing is severely difficult all of a sudden, even with daily use of Ventolin and Spiriva. Are there any other medications you can suggest other than oxygen?
Brian_Southern,_MD: I’m sorry to hear you are not feeling well. Before I would feel comfortable suggesting treatment options, I would need to know more about you and the scar tissue in your lungs, such as how much scar tissue is involved, what does its pattern look like on imaging, what is your pulmonary function. These are the things that would give me a better idea of what type of scarring you have and whether it is a progressive disease.
Nanat426: Good morning, I have sarcoidosis involving my lungs, lymph nodes, bone/marrow and skin. Also SFN. Humira improved the marrow and lungs, but I stopped Humira and now my lungs seem worse. Do you have any suggestions on further treatment? I can't use Imuran or Remicade, and can take prednisone for short boosts only. Currently, I’m only on gabapentin 1500 mg QD, Flexeril 5mg at bedtime, Pulmicort 0.5 bid and Vitamin B12 1000mg q.mo, along with oxycodone for breakthrough pain and ProAir when needed. Thanks so much for your input.
Brian_Southern,_MD: I’m sorry to hear you have had such a complicated course. Imuran, Remicade and Humira are definitely the go-to drugs for someone with multi-organ involvement like you have. Methotrexate is usually one of the first-line agents and is effective if you have not tried this option. We often use other agents such as leflunomide or even rituximab in cases like yours. In summary, there are indeed other options that could be explored.
sarcqueen: I am concerned about side effects from being on prednisone for a long time (especially weight gain and skin problems). For how long can a sarcoidosis patient take prednisone? Does the dose matter?
Brian_Southern,_MD: You are correct to be concerned, as long-term prednisone has numerous side effects. There is no definite time limit for treatment with prednisone, but certain disease treatments may involve up to one to two years of prednisone. Here at Cleveland Clinic, we try to taper the prednisone as quickly as possible and utilize "steroid-sparing" agents whenever we can. These are drugs like methotrexate, azathioprine, leflunomide, etc. that allow you to continue to suppress the immune system without all the side effects of steroids.
Jsweetie: Is it okay to take allergy shots if you have IPF? I am allergic to household dust, mold, pollen, trees, etc. Thank you.
Brian_Southern,_MD: There is no contraindication to receiving allergy shots if you have IPF.
GrannyG: Is there a relationship between arthritis and ILD? I have both and they are getting worse as I get older. It's hard to be very active when my knees hurt and I can't breathe well.
Brian_Southern,_MD: Good question. There is not a known relationship between the most common form of arthritis (osteoarthritis) and ILD. However, there is a strong association of ILD with rheumatoid arthritis and other "autoimmune" types of arthritis.
alg33n: I have allergies and sarcoidosis. I really struggle with sneezing, itching and coughing. My doctor says to just take an over-the-counter allergy pill, but it doesn’t seem to help. Since I have both conditions, could they make each other worse? I feel miserable most of the spring through fall.
Brian_Southern,_MD: Absolutely. Post-nasal drip from allergies can stimulate coughing, which can certainly make your coughing worse. Additionally, many people with allergies have hyper-reactive airways or asthma, which can cause significant complications in sarcoidosis.
Jsmom: I have IPF and my daughter has asthma. Is asthma a risk factor for IPF? Is she more likely to have IPF because I do?
Brian_Southern,_MD: Asthma is not a known risk factor for IPF. However, there is a genetic component to IPF that can run in families. At this time, we do not know enough about the genetics to be able to screen children of patients with IPF. We do recommend that family members of patients with IPF try to avoid things that have been associated, such as smoking.
karenb23r: I was diagnosed with IPF about 12 years ago. I’m interested in exploring a lung transplant since my medication isn’t giving me the best results. My doctor says not to go down that path. How do I know when that’s something to explore? I’m 62.
Brian_Southern,_MD: That is a very good question. Determining whether you would be an appropriate candidate for lung transplantation is a complex process. Your doctor may be taking into account other factors, such as other health conditions you may have that would result in complications during/after transplant. Your age is certainly not a barrier for a transplant. If you are interested in exploring further, I would recommend getting another opinion at a center that evaluates candidates for lung transplants.
byjoel: My doctor wants a lung biopsy. It sounds scary. How do they do that procedure? Is it a major surgery?
Brian_Southern,_MD: Good question. I am assuming he is recommending a surgical lung biopsy. Lung biopsies can also be performed during a bronchoscopy, which is an outpatient procedure. However, for certain diagnoses, bronchoscopic biopsies are not adequate, and the doctor may recommend a surgical lung biopsy. This is indeed a major surgery, but it is usually performed thoracoscopically, which is much less invasive than an "open" lung biopsy. Using a small camera through a few small incisions in the chest, the doctor takes several samples of lung tissue. This collapses the lung and requires placement of an external chest tube until the lung re-expands. Most patients tolerate this procedure very well and are out of the hospital within two to three days. However, there are risks of complications, which should be carefully discussed with your doctor and the surgeon performing the procedure.
Seeking Expert Advice
byjoel: I’m in the process of being diagnosed with pulmonary fibrosis; my physician is trying to figure out which one. I understand determining which one you have can be difficult. Do you recommend getting a second opinion once I’m told which one? I just don’t want to be misdiagnosed and started on the wrong treatment plan.
Brian_Southern,_MD: Great question. I always recommend considering a second opinion in my patients who were recently diagnosed with interstitial lung disease/pulmonary fibrosis. Determining the exact diagnosis is complex, and the diagnosis you receive can affect how you are treated, as well as the prognosis. These are very important factors, and I would want to make sure I felt very confident that I had been diagnosed correctly.
wegwomn: I’m 59 and have vasculitis, Granulomatosis with Polyangitis (GPA or Wegener’s), and just recently it’s starting to affect my lungs. Do you offer any clinical trials for this? Prednisone gives me bad side effects, and I’d like to see if there’s anything else out there for me.
Brian_Southern,_MD: There are a number of ongoing clinical trials looking at the effectiveness of drugs like rituximab in Granulomatosis with Polyangitis (GPA or Wegener’s). The Vasculitis Center here at Cleveland Clinic is often involved in a number of those trials. I would recommend contacting them to see what is currently available. The Center for Vasculitis Care and Research can be reached at 866.275.7496 (toll free).
jacie64: Your ILD center seems great. It’s nice to know all the specialists work together. Are you accepting new patients? I have scleroderma. I would appreciate a second opinion and review of my treatments to see if you and your colleagues would recommend anything different.
Brian_Southern,_MD: Thank you. We think it is great also. We are indeed accepting new patients. We have excellent doctors who treat scleroderma, especially if there is lung involvement. One of our physicians here, Dr. Kristin Highland, is board certified in pulmonology and rheumatology and is an excellent scleroderma physician.
Living with Lung Disease
byjoel: Can pulmonary fibrosis turn into lung cancer?
Brian_Southern,_MD: Good question. This depends on what has caused the pulmonary fibrosis. People with pulmonary fibrosis from certain causes, such as asbestos or silica exposure, are certainly at risk for developing cancer. There have been associations of idiopathic pulmonary fibrosis with cancer, but the link has not been conclusively proven. Most people who develop IPF were smokers at some point in their lives, and smoking is certainly a risk factor for lung cancer.
curlz74: My mother has been diagnosed with IPF and PH. She is at 35 percent lung function and on oxygen at 3 liters. Is there anything we need to keep out of the house or keep away from her if possible, or is it a "try as we go" type of thing?
Brian_Southern,_MD: Good question. I am not aware of any published guidelines or recommendations for things that definitely should be avoided. However, we think that certain things can trigger, or certainly cause, problems in patients with IPF. She should avoid smoke exposure for sure and should try to avoid significant exposure to things like dust as well. The biggest thing to try to avoid, which is not always easy, is exposure to people who have upper respiratory illnesses that can be transmitted.
karenb23r: How can I keep track of my symptoms at home? Should I be testing my lung function regularly?
Brian_Southern,_MD: Great question. Our practice at Cleveland Clinic is to see our ILD patients approximately every three months with pulmonary function testing and other tests as needed. This is a very effective way of following progression of the disease or response to treatment. I often recommend to my patients to purchase a hand-held oximeter (which you can find at any drugstore) and monitor their oxygen saturation periodically. Otherwise, carefully monitoring your symptoms and reporting them regularly to your physician is an excellent way to monitor your lung disease.
br44O: I have sarcoidosis and get flare ups every couple of months. My symptom that is the most difficult to deal with is the chest pain that comes with it. My chest feels tight, and I have a hard time breathing. I get nervous that it’s a heart attack. What do you recommend to help with this pain and scariness?
Brian_Southern,_MD: I would first recommend that your physicians have a good idea about what is causing your chest pain. What you are describing is often related to airway inflammation or hyper-reactivity related to sarcoidosis. This can cause chest tightness or discomfort, difficulty breathing, cough or wheezing. If this is the cause, treatment with inhaler medications can often help.
lynnmarie: My husband is a fireman, and I worry about his lung health greatly. I know the equipment they use has improved, but I still know that he is breathing in some of the smoke, etc. Do you have any recommendations for prevention or screenings?
Brian_Southern,_MD: Usually, these types of occupations will have a screening program or access to a screening program for the development of lung diseases associated with smoke exposure. If not, his primary care doctor should be able to direct him to a screening program, or a pulmonary physician could do some screening tests to see if there have been any effects on the lungs.
MikeOM: We found asbestos in the walls and ceilings when we remodeled our house. How does extended asbestos exposure affect your lungs? Is there a risk for getting ILD?
Brian_Southern,_MD: There is indeed a risk for a certain type of ILD associated with asbestos exposure, called asbestosis. However, this usually requires significant exposure such as those seen in boiler room workers, miners, etc. Unless you were directly working with asbestos fibers, you likely did not have enough exposure to develop asbestos-related lung disease.
Moderator: That is all the time we have for questions today. Thank you, Dr. Southern, for taking the time to educate us about interstitial lung disease.
Brian_Southern,_MD: Thank you all for these excellent questions. I hope you found this chat helpful. Being diagnosed with interstitial lung disease is often scary, as this is a complex group of lung diseases and there is a lot about them we still do not understand. I and my colleagues here at the Cleveland Clinic Interstitial Lung Disease Center and the Sarcoidosis Center of Excellence would be happy to evaluate your particular condition or provide a second opinion. Have a wonderful day!
Moderator: On behalf of Cleveland Clinic, we want to thank you for attending our online health chat. We hope you found it to be helpful and informative. If you would like to learn more about the benefits of choosing Cleveland Clinic for your health concerns, please visit us online at my.clevelandclinic.org.
To make an appointment with Brian Southern, MD, or any of the other specialists in Cleveland Clinic’s Respiratory Institute, please call 216.444.6503, toll-free at 800.223.2273 (ext. 46503) or visit us at clevelandclinic.org/respiratory for more information.
For More Information
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