Online Health Chat with Daniel Culver, DO & Brian Southern, MD
May 17, 2017
Idiopathic pulmonary fibrosis (IPF) is a progressive disease isolated to the lungs. It is a type of interstitial lung disease, which is a group of 200 diseases with similar symptoms but different causes. In IPF, lung tissue becomes scarred. The scarring typically starts at the edges of the lungs and progresses toward the center of the lungs, making it more and more difficult for a person to breathe.
In some patients the disease is genetic (you inherited the disease from your parents). Environmental factors (particularly exposure to certain types of dusts) may also play a role. What is known is that IPF changes the lung's ability to function normally. Typically, mild scarring of the lung tissue occurs first, but over months to years, the normal lung tissue is replaced by more heavily scarred lung tissue, which makes it difficult to breathe and deliver needed oxygen to the body. Risk factors for IPF include working around dust or fumes. Farmers, ranchers, hairdressers, stone cutters/polishers and metal workers face a moderately increased risk of developing the disease due to exposure to these dusts or occupational fumes.
Currently, more than 80,000 adults in the United States have IPF, and more than 30,000 new cases are diagnosed each year. Because lung tissue scarring results in an inability of the lungs to deliver needed oxygen to the body, strain is put on the right side of the heart. This may lead to high blood pressure in the lungs, a condition known as pulmonary hypertension. IPF is also associated with heart attack, respiratory failure, stroke, blood clots in the lung (called pulmonary embolism), lung infections and lung cancer.
About the Speaker
Daniel Culver, DO, is a staff physician in the Department of Pulmonary, Allergy and Critical Care Medicine at Cleveland Clinic's main campus. His clinical interests include sarcoidosis, pulmonary alveolar proteinosis, critical care and interstitial lung diseases.
Dr. Culver earned his Doctor of Osteopathic Medicine degree at the Ohio University College of Osteopathic Medicine in Athens, Ohio. After medical school, he did an osteopathic rotating internship at Meridia SoutePointe Hospital. He completed his residency in internal medicine and a fellowship in pulmonary and critical care medicine at Cleveland Clinic. Dr. Culver is a fellow of the American College of Chest Physicians and a member of several professional associations, including the American Thoracic Society, the American Osteopathic Association and the World Association of Sarcoidosis.
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 Idiopathic Pulmonary Fibrosis
Causes and Confirmations
Evelyn912: I have been diagnosed with IPF because there are enough symptoms to make for a strong possibility what I have is IPF. I chose not to do the VATS test due to risk factors at almost 70 years old and elected to go on medications. It's never too late. Would it have been wiser to do the VATS test to make sure I am on the right course for a confirmed diagnose and on the correct medications?
Daniel_Culver,_DO: Hello, everyone. Thanks for joining the webinar today. Let's start with this question, since it has to do with diagnosis. In general, symptoms of shortness of breath and cough are consistent with the diagnosis of IPF, but they are not required to make the diagnosis. With more CT screening occurring, some patients without symptoms are being diagnosed nowadays. Not everyone with IPF requires a VATS (surgical) lung biopsy. When the high-resolution CT scan shows a very characteristics pattern, and the clinical situation, history and exposures fit, a confident diagnosis of IPF can be made with more than 95 percent accuracy without going forward with a biopsy.
mk004: Can excess radon gas in one's home lead to IPF?
Brian_Southern,_MD: As far as I know, there is no established relationship between radon and IPF. However, radon is the second leading cause of lung cancer in the United States.
Ellavin: Can you please discuss the role of fibroblastic foci in diagnosis and progression? I have one VATS interpretation of focal organizing pneumonia and a second interpretation of fibroblastic foci.
Daniel_Culver,_DO: Fibroblastic foci (FF) are small collections of cells with characteristics of fibroblasts and of muscle cells, so called myofibroblasts. They are found near the leading edge of the fibrotic zone in IPF. There are some data supporting the idea that patients with more numerous FF do tend to progress along more quickly.
FF can be found in other scarring lung diseases, too (though not usually a lot of them), so finding FF does not "prove" you have IPF. We are always more comfortable, however, with making the diagnosis when we can see numerous FF. Focal organizing pneumonia is not really a disease. It may be a reaction of a small area of the lung to some stimulus or injury. To make the diagnosis of IPF pathologically, one must interpret the broad pattern across a larger anatomic area; focal findings are rarely helpful. In this case, I bet that is may be a red herring.
Karen0911: I am a 63-year-old female diagnosed with ILD last year. This was discovered when I had a low-density CT screening for lung cancer. A high-resolution CT scan showed minimal scarring in the lung bases. What can I do to prevent this from getting worse? Is this something that will progress regardless of my actions? How quickly can this become worse? Thank you.
Brian_Southern,_MD: Since the introduction of low-density CT screening, there has been increased detection of "interstitial lung abnormalities." We are still trying to understand the implications of this. I would recommend you have an evaluation by an interstitial lung disease specialist who may be able to make a specific diagnosis. The prognosis (how quickly it may become worse) depends on the diagnosis, as there are many different types of ILD with varying prognoses.
Kandyking: Would you say that IPF can be classified merely by being from an unknown cause, or would you say that is must also be characterized by the way it acts (for instance, microscopic IUP, HRCT honeycombing versus ground glass and/or collagen versus inflammation)? My IPF has an inflammatory component, which seems to react to prednisone and shows much more ground glass with traction bronchiectasis with only some honeycombing, and pathologists can't agree on the existence of IUP. I’m just wondering if you think nintedanib or pirfenidone would be helpful in a case like mine. Thank you.
Brian_Southern,_MD: Excellent question. Our current understanding of IPF is that it is a disorder of signaling between epithelial cells and fibroblasts, and that it is not driven by inflammation. We have had numerous clinical trials demonstrating that suppressing inflammation had no effect in IPF. Our best evidence demonstrates that antifibrotics like nintedanib are the best treatment for IPF, while anti-inflammatory medications like prednisone are the best treatment for inflammatory-driven types of fibrosis, like those associated with autoimmune disease.
rbald: A close friend of mine was recently diagnosed with IPF. He said the doctors estimate his life expectancy to be two to four years. He is about 74 years old and had heart bypass surgery several years ago. Is there a "cure" for this? Is there a way to slow the progress of the disease? The description of this chat said that there are other diseases that have similar symptoms. What method is used to confirm a diagnosis?
Daniel_Culver,_DO: This question addresses a lot of questions. Let's start with prognosis. The historical estimates of IPF survival suggest that the average life expectancy after diagnosis is three to three and a half years. However, these data are problematic since they are based on the control groups seen in tertiary centers that were participating in clinical trials, and also were developed before the advent of the new FDA-approved antifibrotic medications. Also, it is important to remember that these values are averages; some patients will do better, whereas others will be worse. In terms of slowing the progress of the disease, there are a number of proposed interventions. The only ones that have really strong supporting evidence are nintedanib (Ofev) and pirfenidone (Esbriet). These both slow the decline of spirometry, which we think is a marker for the progression of the disease. The benefits for other endpoints, like mortality, walking distance, quality of life and rate of exacerbations, are possibly there but not as strongly proven. Other interventions are also probably helpful, including good hand hygiene, pulmonary rehabilitation, vaccinations, treatment of esophageal reflux disease when present (and maybe even when asymptomatic – controversial) and treatment of other problems, such as sleep apnea.
Jslus: Can someone have both asthma and IPF? Is the treatment for IPF the same for someone with both as opposed to someone only with IPF?
Brian_Southern,_MD: People can indeed have both asthma and IPF. The treatments for these diseases are completely different. Treatment for IPF focuses on slowing down the fibrosis with antifibrotic therapy, while treatment for asthma focuses on reducing airway inflammation
IPF wife: I'm interested in the connection between IPF and weight loss. My husband (diagnosed at Cleveland Clinic in 2015 at age 66 and now being seen by IPF specialists near our home) lost more than 40 pounds over a four-month period last year (now weighs 145). He was on Esbriet but had no gastrointestinal symptoms and no loss of appetite, and his exercise program (weights, treadmill, exercise bike, etc.) remained the same. He was tested from top to bottom for possible causes of weight loss, and nothing was found. He has always been athletic (played competitive lacrosse until injuries led him to hang up his cleats several years ago) and has exercised daily for decades. He has not yet been prescribed oxygen and has regained about 10 pounds over the last year. My question is this: Could his body be working so hard to keep his O2 levels up that it is resulting in weight loss? He uses a wrist computer with a finger sensor to track his O2 while asleep. It shows occasional dips below 88, but mostly remains around 90 to 92. Thanks.
Brian_Southern,_MD: Good question. Weight loss is common in IPF, as it is in other chronic health conditions. Additionally, weight loss is a known possible side effect of Esbriet.
Reach of Research
Canti53: There are a number of clinical trials in process regarding treatment/cure for IPF. Which one(s) do you consider the most promising and why? When will the trials conclude?
Brian_Southern,_MD: In my opinion, there are several promising drugs in clinical trials right now. The two that are in the most advanced stages are: 1) BG00011 (formerly STX-100), an integrin inhibitor, and 2) FG-3019, an inhibitor of connective tissue growth factor. The science behind these is very promising, and I am optimistic.
Canti53: What can patients do to speed up the results of promising clinical trials?
Daniel_Culver,_DO: Clinical trials are more difficult now since the ability of trials to show benefits are more difficult. Imagine that you are trying to show how to make your basketball team better. In the past, you never had any practices then you started practicing and the team got better. That would be easy to see in the wins and losses column. But, it is analogous to the first intervention (pirfenidone and nintedanib trials compared to nothing).
Assessment and Action
Canti53: Which Center of Excellence has the most successful transplant program to deal with an IPF patient with a high level of antibodies?
Brian_Southern,_MD: Auto-antibodies in lung transplant is a difficult problem with which all transplant centers are dealing. I do not think there are centers that are more experienced in dealing with this than others. I would recommend being evaluated for transplant at a center that does a high volume of transplants and has the most experience. Annually, Cleveland Clinic is one of the highest volume transplant centers in the country.
Jsweetie: Is Vicks VapoRub safe for IPF patients to use on their chests?
Brian_Southern,_MD: Interesting question. I am not aware of any studies looking at the safety or efficacy of Vicks VapoRub in IPF.
mabel1: Is there some way to treat sarcoidosis naturally? I have been treated with Deflazacort for two years and I must repeat the treatment, but now I have kidney stones.
Brian_Southern,_MD: We have an upcoming webchat on Sarcoidosis: Management & Treatment Options on June 27. Please join us for that discussion.
Ellavin: What testing is performed on a VATS biopsy and lavage at Cleveland Clinic? I had a second opinion, and the material sent was four lonely slides and that's it. They are both prominent ILD centers. It seems that there should be more sophisticated testing done when you give up that much tissue for such an important diagnosis.
Daniel_Culver,_DO: The testing really depends on what the pathologist thinks needs to be done. The standard is to look at the slides with H&E or other basic stains. Additional stains would be performed according to what is seen in the first part. For example, if infection is suspected, special stains for organisms would be done. Sometimes, the pathologists look more closely at certain aspects of the scarring, using things such as trichrome staining or pentachome staining, to allow better visualization of the walls of blood vessels or the distribution/nature of scarring. Diagnosis of IPF (usual interstitial pneumonia is the diagnosis that pathologist make – the clinical information must be combined with it to make it IPF) – requires assessment of a broader geographic area, usually with multiple lung lobes sampled. If the biopsies you had were large and the slides adequate to see the pathology, the diagnosis may be easily made. Four slides sounds a little skimpy, though. I wonder if the biopsy fragment itself was pretty small. The pathologist in the referring center (like Cleveland Clinic) can request that the institution with the actual biopsy send the tissue blocks so they can make more slides to look more extensively.
Ellavin: What is the approach at Cleveland Clinic when seeking another opinion? How comprehensive is the evaluation, and what's the usual time required in residence in Cleveland, a day, a week?
Brian_Southern,_MD: Good question. Many of the patients we see come here for a second opinion. The time required depends on the complexity of the case. For most cases of interstitial lung disease, we can arrange all of the testing and evaluation over the course of one day. The typical testing (if not done already) usually includes pulmonary function testing, ambulatory oximetry, blood work and imaging. If other specialists are needed, we will try to coordinate those appointments in the same day.
ribbon30: What is the difference between IPF and bronchiectasis? I was diagnosed with both conditions a number of years apart. I have a persistent cough. Advair was very helpful. It is now too expensive for me. What else do you recommend?
Brian_Southern,_MD: Good question. There are two broad categories of bronchiectasis. Classic bronchiectasis occurs when the bronchi (airways) are dilated and filled with mucous. It is usually associated with infectious or inflammatory conditions. Traction bronchiectasis occurs when the smaller airways in the periphery of the lungs are pulled open by lung scarring. This is the type of bronchiectasis typically associated with IPF. There is no known role for treatment of traction bronchiectasis.
Ellavin: What is the difference between the fibroelastosis of PPFE and the marked increase in elastin of IPF, NSIP and others? I have airway-centered interstitial fibroelastosis and would like to know more about this pretty rare presentation.
Brian_Southern,_MD: Excellent question. Pleuroparenchymal fibroelastosis is a recently described entity that is similar in presentation and course to IPF. However, the fibrosis typically affects the upper lobes (in contrast to lower lobe involvement in IPF), and the scar tissue contains a significant amount of elastin (in contrast to the scar tissue in IPF that is predominantly collagen). I am aware of one series that has been published on airway-centered fibroelastosis, which is similar to PPFE. As we are still trying to understand these diseases, there have not yet been effective guidelines or therapies published.
ge2010: What is the best way to stay the same once you have been diagnosed with a disease? I have interstitial lung disease - hypersensitivity pneumonitis. What is the best treatment? How can exercise help your lungs to keep them from getting worse? I work out at least 35 minutes a day on a stationary bike or walk two miles on a treadmill.
How do you prevent coughing?
Brian_Southern,_MD: With regard to hypersensitivity pneumonitis, the best known way to prevent progression is to remove the exposure that caused it. In some cases, the exposure cannot be identified or the disease may continue to worsen even once the exposure has been removed. There is some medical literature supporting the use of prednisone specifically in farmers' lung or bird fanciers' lung disease. Many specialists use other immunosuppressant agents in HP with mixed results.
Ge210: If my PFT results are the same as six months ago, would you recommend changing drugs? Can Cellcept help a person’s lungs?
Brian_Southern,_MD: We typically monitor whether IPF is getting worse by looking at pulmonary function testing every three to six months. As IPF is a progressive disease characterized by continued decline in pulmonary function, the fact that your PFT results are unchanged is a sign that your lung disease is stable. I typically do not recommend changing medications if the lung function is stable. Cellcept (mycophenolate) has NOT been demonstrated to be effective in IPF. It is often used in other forms of pulmonary fibrosis, such as those associated with autoimmune disease.
Ellavin: I belong to the Inspire PF community. Some members are taking Esbriet or Ofev, but don't have a diagnosis of IPF. Are the guidelines for prescribing these two drugs changing, and will insurances pay for non-IPF treatment?
Daniel_Culver,_DO: No. Both of those are approved only for IPF, although there are some clinical trials going on looking at using them for other indications where fibrosis occurs. In our experience, these medications are usually only approved by insurers when the diagnosis of IPF is listed, although there are some unique situations where patients appear to be able to get these medications (often after appeal) despite not having IPF. We do not currently really know if the two antifibrotic medications are going to work for other diseases such as NSIP, chronic HP or fibrosis related to autoimmune diseases.
msen: I am on Cellcept for HP, which seems to flare up my UC. Imuran made me very sick. Would an IV-delivered medicine be the next step?
Brian_Southern,_MD: There is not a lot of good evidence to guide therapy in chronic hypersensitivity pneumonitis. Most doctors recommend the drugs you mentioned. We have also had some success using leflunomide. There have been reports in the literature of success with rituximab (an IV medication) in patients who did not respond to these drugs.
Jswee: I understand in studies that NAC didn’t really show any promise for the IPF patient. I also heard from a doctor at National Jewish that, in some cases, it can cause harm. I have taken NAC for almost five years since my diagnosis with no adverse effects. My PFT numbers by percentage are all pretty much over 100 percent. In a case such as this, what brand do you recommend?
Daniel_Culver,_DO: There is a single study that shows that a subset of patients with IPF are harmed, and another subset are benefited, by use of NAC. This depended on a genetic polymorphism for the TOLLLIP gene. Currently, that type of genetic polymorphism testing is not routinely available, and the results of that analysis will need to be validated in a second group before that testing can be used as a "precision medicine" approach for treatment of IPF. I am always reluctant to rock the boat by changing therapies in patients with stable disease, but I do think it is worth a conversation with your health care team.
boyscout2726: I'm Kerry, and I'm 66 years old. I have been taking Esbriet since December 2014, and think it may have slowed the progress of my IPF somewhat. Even so, I dropped 10 percent in forced vital capacity in the last year to 56 percent of predicted. I'm not using oxygen yet and can still exercise. My question: Does research support continued use of Esbriet for people with "severe" IPF? Is it true that when FVC falls below 50 percent of predicted, it is classified as severe? I have read online that many insurance companies will not support Esbriet therapy if your FVC is under 50 percent, because Phase III testing did not include many people in that stage, so there is no hard evidence that it helps. Is this true? Why would it not still help?
Daniel_Culver,_DO: This is a tough area. There is a post-hoc analysis showing that among people whose FVC dropped by 10 percent, the individuals who continued on Esbriet had better outcomes than those who stopped. Some physicians, therefore, continue using Esbriet regardless of decline. In the UK, on the other hand, once you drop 10 percent you are no longer able to use it. The data about continuing Esbriet doesn't give us the answers to the questions of whether continuing Esbriet is better or switching to Ofev would be better. There are also trials occurring looking at combining the two agents. In terms of the notion of using it in those with FVC < 50 percent, it is true that we don't really have firm data on it. I personally don't think there is any biologic reason to believe that the medications would not be effective in those with FVC < 50 percent, so the practice in our group is to offer it to all regardless of PFTs. Of course, in patients with very advanced, end-stage disease, the discussion should center on whether the expected benefits are really worth it, compared to the possible side-effects. In our experience, most insurance companies have continued to allow these medications in those with FVC < 50 percent.
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 http://my.clevelandclinic.org.
Brian_Southern,_MD: Thank you all for participating. We have enjoyed answering your questions. IPF is a difficult disease to deal with, and we are happy to help in any way we can. If you need further information or would like to have a more formal evaluation, please do not hesitate to contact us.
To make an appointment with Daniel Culver, DO, or 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.
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At Cleveland Clinic's Respiratory Institute, we provide world-class patient care by combining our strengths in clinical expertise, research and education. Ranked number three in the country by US News and World Report for pulmonary care, we treat nearly 90,000 patients annually. With more than 100 pulmonologists, allergists/immunologists and critical care specialists, the Respiratory Institute diagnoses and treats a wide range of lung, allergy and breathing-related conditions.
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