Interstitial Lung Disease
October 11, 2013
Interstitial lung disease (ILD) is a group of conditions that cause scarring to 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 interstitial lung disease (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 Speakers
Daniel Culver, DO is a staff physician in Cleveland Clinic’s Department of Pulmonary, Allergy, and Critical Care Medicine. He is board-certified in internal medicine with additional specialty certification in pulmonary medicine and critical care medicine. Dr. Culver’s clinical interests include interstitial lung diseases, sarcoidosis, pulmonary alveolar proteinosis and critical care.
Dr. Culver completed his residency in internal medicine, and a fellowship in pulmonary and critical care medicine at Cleveland Clinic after completing his residency in internal medicine. He did an osteopathic rotating internship at SouthPointe Hospital in Warrensville Heights, Oh after earning his doctor of osteopathic medicine degree at the Ohio University College of Osteopathic Medicine in Athens, Oh. Dr. Culver currently sees patients at Cleveland Clinic Main Campus.
Jihane Faress, MD is a physician in Cleveland Clinic’s Department of Pulmonary, Allergy, and Critical Care Medicine. She is board certified in Internal Medicine, Internal Medicine - Critical Care Medicine and Internal Medicine - Pulmonary Disease.
Her specialty interests include interstitial lung disease, sarcoidosis and critical care medicine.
Dr. Faress completed her fellowship in pulmonary and critical care medicine at University Hospitals of Cleveland, in Cleveland, Oh after completing her residency in internal medicine at Johnson City Medical Center, in Johnson City, Tenn. She also completed an internship in pediatrics at Hôtel-Dieu de France Hospital, in Beirut, Lebanon, following her graduation from medical school at Saint Joseph University Faculty of Medicine, in Beirut, Lebanon. Dr. Faress currently sees patients at Cleveland Clinic Main Campus.
Let’s Chat About Interstitial Lung Disease
Moderator: Welcome to our chat ‘Interstitial Lung Disease’ with Cleveland Clinic lung specialists Dr. Daniel Culver and Dr. Jihane Faress. They will answer questions about ILD including diagnosis and treatment options.
Daniel_Culver,_DO: Good afternoon everyone—we're excited to embark on this topic. Today, we are focusing on interstitial lung disease (ILD).
Nonspecific Interstitial Pneumonia vs. Idiopathic Pulmonary Fibrosis
boyscout2726: Based on a high-resolution CT done in August, I have either early idiopathic pulmonary fibrosis (IPF) or a fibrotic form of NSIP (nonspecific interstitial pneumonia). Blood tests ruled out several other diseases like lupus. I am awaiting the lung biopsy results by flexible bronchoscopy that I had done yesterday. I am 62 years old—and I consider myself fit and pretty healthy other than this. Actually, I am not experiencing any symptoms from this yet. My doctor just happened to see something on a chest x-ray last November. How concerned should I be if it is confirmed to be IPF or NSIP?
Daniel_Culver,_DO: This is an important distinction, since most NSIP is related to some other process—typically a connective tissue disease like scleroderma or polymyositis or others. These may be treated with immune suppressant medications, while IPF may actually be worsened by immune suppressant medications. Sometimes the connective tissue disease may not be apparent yet at the time of the onset of the lung disease. In those situations where there is not a clear-cut connective tissue disease, a surgical lung biopsy may be helpful to clarify the diagnosis. A very rare entity is idiopathic NSIP—that is NSIP that is not caused by any known triggering factor. Whether immunosuppressant medications are helpful for that is unknown, although most experts think that they may be useful for stabilizing the progression of the disease.
Interstitial Pneumonia Diagnosis
Jim123: Is interstitial pneumonia the same condition as interstitial pneumonitis? Do they respond to antibiotics? What is the usual outcome of the conditions?
Jihane_Faress,_MD: We use the two words interchangeably. They do not mean an infection and, thus, they do not respond to antibiotics. The outcome depends on the specific type of the interstitial pneumonia or pneumonitis (IP). Idiopathic pulmonary fibrosis (IPF) is one example of an idiopathic IP that has a bad prognosis.
1958WINTER: My husband has interstitial lung disease (ILD) with a diagnosis of usual interstitial pneumonia (UIP). He has had biopsies and breathing tests. They say this is what he has, but as of right now there will be no treatment. As his disease progresses, they will treat symptoms only and then when he is in last stages maybe he can get on a transplant list somewhere. Is this the usual treatment for UIP? They will not tell us what stage he is in. He will have a breathing test every six months, and then they can tell us how fast his fibrosis is progressing. He had CT scans in 2010 and 2013. Couldn't they tell how fast it is progressing from those scans? Also, my husband does home remodeling. I asked it is was alright for him to tear out walls and paint and mow etc. They said he did not need to be around irritants, but he could go ahead with this work while wearing a mask. I am not sure about that either.
Jihane_Faress,_MD: I am assuming your husband has a surgical lung biopsy showing UIP. The first thing to know is that not all UIP are idiopathic pulmonary fibrosis (IPF). I will answer the question as if he has UIP IPF. It is true that there is no proven therapy that could prevent disease progression or increase survival in IPF. But there are other things that we offer patients.
- It is crucial to confirm that the diagnosis is IPF and not UIP related to other systemic diseases, exposures, chronic hypersensitivity pneumonitis or medication. A confident diagnosis can be made by having a multidisciplinary discussion in an academic center that specializes in interstitial lung disease.
- For IPF we offer patients enrollment in clinical trials depending on their age and their degree of pulmonary limitation. We have a few promising drugs that are currently being studied.
- We typically can tell how fast patients are progressing by looking at their pulmonary function tests. if their FVC (forced vital capacity) is declining more than 10 percent over six months, then we should consider referral to lung transplantation evaluation. Assuming they are 65 years old or younger.
- There are a lot of occupational exposures that are thought to be risk factors for IPF. These include farming, woodworking and metal dust exposures. I would recommend that your husband wears a mask.
Interstitial Lung Disease Diagnosis
I L D Wifey: My husband has recently been diagnosed with interstitial lung disease (ILD). Is it necessary for him to have a biopsy to determine which type he has, or does that really matter? Can he just be treated according to symptoms rather than going through this invasive procedure? (They also found a 1 cm spot on the left lobe.)
Jihane_Faress,_MD: A lung biopsy is not always necessary. Sometimes a chest CT scan, when done with the right technique, (high resolution chest CT) can make the diagnosis of idiopathic pulmonary fibrosis (IPF) with a high degree of confidence and save the patient from an invasive procedure. Having said that, it is important that the radiologist who interprets the scan is experienced in reading interstitial lung disease. Regarding the spot in the lung, they can do additional tests to try to differentiate if it is malignant or benign such as a PET scan, and decide the need to resect it based on the results.
pdl1948: What's the difference between idiopathic and interstitial lung disease?
Jihane_Faress,_MD: The interstitial lung diseases (ILDs) are a group of disorders that share similar clinical, radiographic and physiologic manifestations. The descriptive term ‘interstitial’ means that they affect a part of the lung called pulmonary interstitium. We prefer to use the term ‘diffuse parenchymal lung disease’ over interstitial lung disease because the majority of these disorder affect, in addition to the pulmonary interstitium, the alveolar sacs and the small breathing tubes.
Idiopathic means without an identifiable cause. For example idiopathic pulmonary fibrosis (IPF) is a form of ILD without a known cause.
Interstitial Lung Disease and Pulmonary Function Tests
oktriath: I am a 70-year-old woman who was diagnosed with pulmonary sarcoidosis 13 years ago. The pulmonary sarcoidosis resolved. My symptoms of acute dyspnea were in remission until this summer. I am typically very active as a former triathlete and cyclist. During a seven to ten day fever of 102 to104 degrees early in July, I developed shortness of breath (SOB). I was diagnosed with a urinary tract infection (UTI) that resolved with course of Bactrim® (trimethoprim and sulfamethoxazole). Since the episode of fever I have had symptoms of SOB with exertion and feeling that I can't take a deep breath. The pulmonary function tests were done in September. My pulmonary functions tests are o.k., yet I feel SOB with exertion and unable to take a deep breath. I have some documented fibrosis. What should I do next?
Daniel_Culver,_DO: Pulmonary function tests (PFTs) are only one part of the story. First, the numbers on the PFTs are only based on population averages. It would be like saying that basketball player Kareem Abdul Jabaar shouldn't feel short if he came in measuring five foot and 11 inches tall. Of course, that is not ‘normal’ for him. So, the first issue is where did you start? More important than any absolute number is the rate of decline (change) in the function tests. We also consider the degree of dyspnea, the imaging features, the need for oxygen and the effects on quality of life when we determine how severe the disease is. The serial (longitudinal over time) tests are the most important for providing information. When the FVC (forced vial capacity) drops by five to 10 percent, it is considered a sign of high risk for future trouble, including death. What should be done next depends on which particular interstitial lung disease (ILD) you have.
Interstitial Lung Disease and Mold
JE: I have been diagnosed with interstitial lung disease (ILD) with no more specificity although it is believed to be hypersensitivity pneumonitis. (I have had a biopsy, radiographs, blood tests, etc.) My symptoms have generally subsided after a significant drug protocol. I am trying to get my home and workplace tested for environmental issues in case that is the cause. Are there suggestions of specific things to test for besides mold, mildew and gasses? I am not around birds, but we also are exploring the possibility of down comforters and pillows.
Daniel_Culver,_DO: Experts' opinions are all over the place on this. I think that home testing is really tricky because there are always molds found. I get interested in ‘What can I do about it?’ questions. Unless you have a positive HP serology (blood test), it matches up with a place in your home where there is mold, and it has been verified as that mold on testing—there is little you can do. For example, let's say your home has some molds like Alternaria and Cladosporium that were found on air testing. What are you going to do about it unless there is a place somewhere in your home that has visible mold growth that can be abated (fixed)? In that case, I would just go ahead with the repairs anyway since the blood tests are not very reliable. In the case of feather pillows and comforters—if the blood tests show significant elevation about the normal range—not just a tiny bit, I think it is best to get rid of feather-containing objects in your house. However, there is not much literature anywhere to support that doing any of these strategies makes a difference if the HP is chronic.
Pulmonary Fibrosis Diagnosis
VEAUHADI: How do you confirm that a patient has pulmonary fibrosis? I have read that treatments only offer limited help and do not stop pulmonary fibrosis. Is this correct?
Daniel_Culver,_DO: It is important to realize that pulmonary fibrosis and idiopathic pulmonary fibrosis (IPF) are not the same. Pulmonary fibrosis means scarring of the lung. There are over 100 causes of pulmonary fibrosis. Some of these causes are relatively benign. It is important to be seen at a center with experience in order to sort through a particular case. IPF is the most common cause of pulmonary fibrosis, but still is the minority of all the cases of patients with lung scarring.
Hottomale: Has Prozac® (fluoxetine hydrochloride) been linked to causing fibrosis?
Jihane_Faress,_MD: While a definite link does not exist, there are case reports of SSRI (selective serotonin reuptake inhibitor)-induced hypersensitivity pneumonitis and eosinophilic pneumonia.
Idiopathic Pulmonary Fibrosis Prognosis
Jim123: Is this type of lung condition always progressive—or can it be kept stable for many years or even decades?
Jihane_Faress,_MD: Idiopathic pulmonary fibrosis (IPF) is a progressive disease. Some patients progress slowly over years. Others will be stable for some time, but can have a severe rapid decline which indicates an acute exacerbation (AE). The AE does not respond to treatment, and leaves the patient with much worse lung function and worse dyspnea.
Hottomale: What is the progression difference between post-inflammatory pulmonary fibrosis, chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF)? Are they all ultimately the same with a ‘three-to-five-year-until-death’ prognosis?
Jihane_Faress,_MD: Post-inflammatory pulmonary fibrosis is a term used broadly to describe different types of pulmonary fibrosis that are not idiopathic. It can be seen in the setting of connective tissue diseases among other diseases. It is thought to have a better prognosis than IPF. The prognosis of COPD depends on the stage of the disease.
Restrictive Lung Disease
CurlsForLife: In 2011, my husband was told by the local Veterans Health Administration hospital that he has restrictive lung disease, but they did not perform a biopsy to determine a specific diagnosis. His health fluctuates so much—his breathing, energy level, and blood pressure. It will appear that he is on a downhill spiral and then he bounces back. Is this normal?
Jihane_Faress,_MD: Restrictive lung disease is a term used to describe small lung volumes. It does not tell anything about the cause. There are two groups of disorders that cause restrictive lung disease. One does not involve the lungs per se, but what is surrounding the lungs such as the muscles and adipose tissue. Examples of these would be myopathy (muscular weakness) or obesity. The second group that affects the lung itself is the interstitial lung diseases.
Gastroesophageal Reflux Disease (GERD) and Lung Disease
derekl: Has a connection been made between GERD (acid reflux disease) and lung disease?
Jihane_Faress,_MD: There are some studies that link reflux and micro-aspiration to pulmonary fibrosis, especially the idiopathic form. Reflux might be a risk factor to acute exacerbation of idiopathic pulmonary fibrosis (IPF). In my practice, I am very aggressive in treating GERD in patients with IPF.
boyscout2726: I have severe gastroesophageal reflux disease (GERD) that has not responded to drug therapy. My early idiopathic pulmonary fibrosis (IPF) is diffused throughout my lower middle and upper lungs. I am told that if GERD was exacerbating it, all the damage would be at the base of my lungs. Is this true?
Jihane_Faress,_MD: GERD has been linked to pulmonary fibrosis in a small retrospective case series study. GERD and silent aspiration are thought to be risk factors for acute exacerbation (worsening) of IPF. Lastly patients who have asymmetric IPF (where one lung is more involved than the other) are more likely to have GERD.
moana329: I have idiopathic pulmonary fibrosis (IPF), but I am not on oxygen yet. I have asked my doctor about pulmonary rehabilitation, but he has told me I don't need it yet and won't refer me. I exercise on my own, but it seems like there is a lot that I could gain by being a part of a rehabilitation program—access to information, to breathing techniques, nutrition information, to mutual support, etc. I want to fight this disease in every way I can. Wouldn't it be very beneficial to attend rehabilitation? Is this typical for rehabilitation programs to limit access to only those who are severely debilitated by this disease?
Jihane_Faress,_MD: Pulmonary rehabilitation is a great tool for muscular conditioning in a variety of chronic pulmonary disorders including IPF. The patients who benefit most from it are those who have moderate-to-severe pulmonary limitation. I would encourage you to continue to exercise on your own. And depending on your pulmonary function limitation, even if you are not on oxygen, you might qualify to pulmonary rehabilitation.
Francis: Would pulmonary rehabilitation help my overall lung function?
Daniel_Culver,_DO: Not much, if at all. The main benefits relate to improvements in the cardiovascular and muscular systems, so that the burden on the lungs during walking or exercise is lowered—thus helping with shortness of breath and ability to perform work, walk, etc.
Treatment for Cough Control
devojka: My 75-year-old husband was diagnosed with interstitial lung disease (ILD), two years ago, due to working as a bricklayer with exposure to asbestos and cement dust. He coughs quite a lot, which is productive with clear mucus. He takes no medications for ILD, and no oxygen. His pulmonary function test and CT scans show a slow decline each time. Is there anything that would help him with the cough to get rid of the mucus easier? Would a mask help with the colder weather?
Jihane_Faress,_MD: For a cough that is related to pulmonary fibrosis, we have some success using a codeine-based syrup. We also need to rule out other common causes of cough that are not necessarily related to the fibrosis, such as reflux, post-nasal drip and chronic obstructive pulmonary disease (COPD), since they are treated differently.
Bkjenks: I received a second opinion for the diagnosis of interstitial lung disease (ILD). I started on 30 mg prednisone in May until being gradually taken off in early October. That treatment helped with symptoms, but the side effects were not good. Since being off prednisone I have less energy, the cough is worse, and more oxygen is required than prior to prednisone. Is there anything other than cough drops to ease a cough? What treatments are available at this time other than oxygen therapy? What side effects can be expected if scarring worsens?
Jihane_Faress,_MD: When it comes to treating cough it depends on the cause of the cough. For a cough due to lung inflammation, prednisone can help by treating the underlying disease. When lung scarring is the main issue, prednisone might not have an effect on the cough. We sometimes use codeine-based medication such as Hycodan® (hydrocodone bitartrate and homatropine methylbromide) syrup to treat cough. Codeine is a centrally acting anti-cough agent. When prednisone leads to an objective improvement but the side effects are prohibitive, we recommend the use of other immunosuppressive medication. When the scarring worsens, there is typically worsening of dyspnea, cough and also an increase in the oxygen needs.
Idiopathic Pulmonary Fibrosis Treatment
arkie988: With a diagnosis of idiopathic pulmonary fibrosis (IPF), we feel frustrated about waiting for scans every six months. What, in your opinion, is the best course of action when newly diagnosed? Are there every day actions a patient can take to improve their quality of life, even down the road? I am thinking dietary changes, exercise, etc. We just need some more advice and direction.
Jihane_Faress,_MD: If the diagnosis of IPF is secure, we typically recommend the following:
- Offer enrollment in clinical trials.
- Prescribe supplemental oxygen with activity if indicated
- Pulmonary rehabilitation
- Gastroesophageal reflux disease (GERD) treatment
- Update vaccination against Streptococcus pneumonia and influenza
- Screen for pulmonary hypertension and consider treatment
- Screen for sleep disordered breathing and treat accordingly
Popho65: I have been on prednisone for several years. One doctor says it doesn't help and wants me off of it. The side effects are not good. Is it possible to get off of it and how? I am on 25 mg now but have been on as low as 10 mg. I started three months ago with Imuran® (azathioprine or AZA) 50 mg but now I am on 100 mg.
Daniel_Culver,_DO: This is a really tough situation. I assume you have idiopathic pulmonary fibrosis (IPF). If you have a different disease, then immune suppressant medications may be frequently used. But, in the case of IPF, your situation is controversial. The NIH PANTHER trial showed that starting patients with IPF on prednisone plus AZA led to more deaths than using no medical therapies. However, that doesn't necessarily mean that individuals who are already on those medications are best served by stopping them. The available data and your particular situation should be discussed carefully with your treating doctor. There is no exact agreement on this issue in the IPF experts’ community, in large part because there are no substantial data for your situation.
Interstitial Lung Disease Treatment
Snowgoer: I have interstitial lung disease (ILD). I am on two and one half liters of oxygen, which is fine. I was using a breathalyzer with albuterol and budesonide. Should this be continued or are there other more acceptable substitutes? Are there any new and approved medications for treating the inflammation and scarring (fibrosis) of alveoli in pulmonary fibrosis disease?
Jihane_Faress,_MD: Albuterol is a bronchodilator and budesonide is an inhaled steroid. These types of medications work in asthma and chronic obstructive pulmonary disease (COPD), but they typically don’t work in pulmonary fibrosis. Please refer to previous question regarding treatment for idiopathic pulmonary fibrosis (IPF).
Interstitial Lung Disease and Sjögren Syndrome
cordelia: Is the usual treatment for interstitial lung disease (ILD) with pulmonary fibrosis related to Sjögren syndrome? I am being monitored with pulmonary function tests (PFTs), chest x-rays, and a visit to a pulmonologist every three months. I use a Flovent® (fluticasone propionate) inhaler and take Prilosec® (omeprazole) twice daily for gastroesophageal reflux disease (GERD). I have a frequent cough with small amounts of mucus production. I did not have a lung biopsy. Also is it possible that my minimal symptoms will stay the same or will they definitely get worse?
Jihane_Faress,_MD: Management strategies for Sjögren-associated lung diseases are empiric, meaning no controlled studies have been performed. We base the treatment approach on the specific lung pathology, and the severity of symptoms, pulmonary function tests impairment and extent of radiographic disease. When symptoms are mild with minimal PFT limitation, it is not uncommon to monitor longitudinally as the disease can stabilize on its own. If progression is noted, then immune suppressive medications can be prescribed.
Immune Suppressive Treatment
Francis: I was diagnosed with interstitial lung disease (ILD), diabetes mellitus (DM), and polymyositis (PM) in March 2012. I take CellCept® (mycophenolate mofetil) 2500 mg and Neurontin® (gabapentin) 900 mg daily. Do you think my present lung function of 58 percent capacity can improve even if I have fibrosis of the lungs? My next function tests are coming up in November. My physician said that if they aren't improved, he would like me to see the physicians at Cleveland Clinic. Do you have any more suggestions for improvement? I still have flares and problems with stamina, but feel quite better than late last year.
Jihane_Faress,_MD: Not all types of fibrosis are similar. The type of fibrosis that is typically associated with DM and PM is more likely to respond to immune suppressive treatment than the idiopathic forms. So, yes, your lung function could improve. If it does not, there are other medications that we can try.
Pulmonary Hypertension Treatment with Idiopathic Pulmonary Fibrosis
pnmdirect: Is there a preferred drug to treat pulmonary hypertension? If you have idiopathic pulmonary fibrosis (IPF) and no other lung issues, is Advair® (fluticasone propionate) helpful?
Jihane_Faress,_MD: Advair® is a combination of bronchodilator and inhaled steroids that works in asthma and chronic obstructive pulmonary disease (COPD), but not in IPF. For the treatment of pulmonary hypertension in IPF, Revatio® (sildenafil) is the preferred treatment because it doesn’t worsen oxygenation.
moosdapper: I am 72 years old, and have idiopathic pulmonary fibrosis (IPF). I am taking Esbriet® (pirfenidone) 1602 mg and prednisone 7.5 mg daily. Can Acirca® (tadalafil) 20 mg be taken occasionally?
Daniel_Culver,_DO: There have been some studies looking at treating pulmonary elevated blood pressure (pulmonary hypertension) in IPF patients. These studies are directed at either theoretical effects of some of the agents (e.g., endothelin), or the attempt to lower pulmonary blood pressure.
Mostly, these trials have not shown a benefit for pulmonary hypertension treatment. However, in a subgroup analysis of sildenafil (similar to tadalafil) in IPF patients in the STEP-IPF trial suggested that those with abnormal echocardiograms (which suggests more severe pulmonary hypertension) may benefit from this type of therapy. (Revatio® is the brand name for sildenafil.)
Treatment for Chronic Hypersensitivity Pneumonitis
jweppner: I was diagnosed with hypersensitive pneumonitis (HP) about four years ago, and my total lung capacity is around 50 percent of normal for my age. I am a 68-year-old Caucasian male. I have seen prominent pulmonologists at both Mayo Clinic and Northwestern Memorial Hospital. They cannot find a way to stop the ongoing decline in my lung capacity. I am currently taking methotrexate.
Daniel_Culver,_DO: If you have chronic hypersensitivity pneumonitis with substantial scarring on your CT scan, it can be very difficult or impossible to stop the progression, unfortunately. We usually aim for stability with not much improvement in this situation. There are some other agents we typically use for chronic HP, including Imuran® (azathioprine or AZA) and Arava® (leflunomide). We also have some experience now using Rituxan® (rituximab) for HP. All of those may be viable alternatives.
kd3rf: I have been diagnosed with idiopathic pulmonary fibrosis (IPF) and have gone through all the ‘usual’ tests including lung biopsy, pulmonary rehabilitation and transplant evaluation. At the present I can maintain 94 to 96 percent on room air, but my SpO2 (oxygen saturation) drops rapidly into the mid to low 80s with even mild exertion such as walking up a flight of stairs. My pulmonologist says my PFT's are ‘borderline.’ I am wondering at what point does transplantation become a necessity?
Daniel_Culver,_DO: This relates to the timing of the decision to list for transplant. That decision has to be based on the speed of time from evaluation to average transplant for the center you are at, the speed of your progression, and the severity of the disease right now. When patients start to require high oxygen flow with walking and some oxygen at rest, we usually consider that a time to proceed with transplant evaluation.
Gramps195: I am 71 years old, and am in good physical condition. I have had three mild strokes followed by a patent foramen ovale (PFO) closure several years ago. Recently, I have been diagnosed with mild-to-moderate idiopathic pulmonary fibrosis (IPF). Is there an option for a lung transplant in my future?
Daniel_Culver,_DO: Lung transplant is a good option for those who need it, but the 5-year survival median is still not ideal—probably around five and one half to six years. So, we defer transplant until the situation is fairly severe. Although many transplant centers have an age cut-off of 65 years, not all do. Some go by biologic age (e.g., ‘a young 68-year-old’) rather than chronological age. Our center approaches it like that. However, 71 years old is near the top age. If you are not near the transplant window, it would probably not be in your best interest to pursue it right now. Your IPF may remain fairly stable, so you would not want to rush into such a big procedure and commitment unless you are really in a place where you have to. The PFO part sounds like it is the cause of your strokes and fixed. I doubt that would be a roadblock.
wescotte: Are there any interesting new drugs for idiopathic pulmonary fibrosis (IPF) on the horizon?
Daniel_Culver,_DO: Our group thinks that this is a very exciting time for IPF therapies. There are three medications that are in late-stage trials in the U.S.: N-acetylcysteine, pirfenidone, and neratinib. All of these have earlier trials that suggest they may be beneficial, and, as you may know, pirfenidone is approved in many other countries for IPF. There are many medications in phase 2 trials. We are involved in several of these, based on which ones we think have a very strong chance of being useful. Many of these are based on years of laboratory study focused on IPF, and have quite strong biologic rationale. Many of them are targeting a new paradigm in IPF, i.e. ‘scar begets scar.’ So, they are intended to interrupt the formation of the scar tissue itself, not just the cells that cause scarring, which may be useful.
skh727: I read about a clinical trial on N-acetylcysteine (NAC) and its effect on interstitial lung disease (ILD). Do you know if this is so, and the findings of the trial?
Daniel_Culver,_DO: There is one major published trial (IFIGENIA) looking at NAC in idiopathic pulmonary fibrosis (IPF). It compared NAC with prednisone and azathioprine (AZA) versus prednisone and AZA alone. It found that those patients who got NAC did better in terms of progression of disease. However, there was no group getting NAC as a stand-alone therapy, so the results are difficult to interpret. More recently, the NIH-sponsored PANTHER trial showed that prednisone and AZA alone is harmful, leading to more deaths than placebo. (The brand name for AZA is Imuran®) Another arm of that trial just completed follow-up recently, and we should know the results next year. That will attempt to give us an answer to the question of whether NAC taken as a monotherapy is useful. Stay tuned.
Not breathing easy: Using thorancentesis, I was diagnosed with mild nonspecific interstitial fibrosis and emphysematous changes in October 2011. I use oxygen at night and take n-acetylcysteine 600 mg three times daily. I never smoked or had been around smoking. One pulmonologist prescribed prednisone, but the University of Alabama in Birmingham said it is not beneficial. What are your thoughts about the benefits of prednisone? In your opinion, are there benefits to adhering to an anti-inflammatory diet in regard to idiopathic pulmonary fibrosis (IPF)? Have been there any significant advances toward stem-cell therapy in the United States?
Jihane_Faress,_MD: There is a clear evidence that prednisone is harmful for patients with IPF and should not be used. Indeed, the recent PANTHER study showed that prednisone and azathioprine (AZA) (Imuran®) increase mortality in patients with IPF.
Since the current thinking is that IPF is not an inflammatory disease but more a wound healing problem, I don’t think that anti-inflammatory diet would be of benefit.
Stem cell research in pulmonary fibrosis looks promising. They noticed in cardiac trials with intravenous administration of mesenchymal stem cells, the cells would always get deposited in the lung first and differentiate. The University of Miami just got FDA approval to start a phase 1 trial in IPF.
Jsweetie: If perfinidone is approved by the FDA after this phase 3 trial, what is the earliest possible date we might expect that it would become available to patients in the United States?
Daniel_Culver,_DO: That is a difficult question, as the timing of the application to the FDA and the speed of their response are unknown. However, based on the follow-up period of the study, I might speculate that it could be available sometime in the latter half of 2014, if nothing interrupts the process (assuming the trial is positive).
Glenlary: Do they do clinical studies with patients?
Moderator: Clinical trials are research studies that explore whether a medical strategy, treatment, or device is safe and effective for humans. For clinical trials, please visit www.clinicaltrials.gov.
Glenlary: Does Cleveland Clinic do clinical trials?
Daniel_Culver,_DO: Yes, we currently have four active trials for idiopathic pulmonary fibrosis (IPF) at Cleveland Clinic.
belvedere73: I am a 74- year-old woman who has been diagnosed with interstitial lung disease (ILD) and pulmonary fibrosis. Since being diagnosed two years ago my total lung capacity started at less than 75 and is now less than 79. Using an oximeter daily it shows 98 percent. I walk four to five miles daily. My FEV1 (forced expiratory volume 1)/FVC (forced vital capacity), or Tiffeneau-Pinelli index, is 112. Can I be doing anything that would help my situation?
Jihane_Faress,_MD: Not all diagnoses of interstitial lung disease mean idiopathic pulmonary fibrosis. And not all interstitial lung disease have the same prognosis. The rate of progression depends on the specific pathology. Idiopathic pulmonary fibrosis (IPF) is the worse diagnosis. Other ILD can remain stable over time. Having said that, we recommend that people with ILD or other chronic respiratory illnesses participate in exercise programs to prevent deconditioning.
Moderator: Thank you for your questions. We appreciate your participation and hope you will join us for other chat topics in the future.
Daniel_Culver,_DO: Thank you very much for your interest and participation. We will schedule another chat soon.
To make an appointment with Dr. Culver, Dr. Faress or any of our specialists in the Respiratory Institute, please call 216.444.6503 or 800.223.2273, ext. 4650. You may also visit us online at www.clevelandclinic.org/respiratory.
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