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Interstitial Lung Disease

Online Health Chat with Daniel Culver, D.O. and Joseph Parambil, M.D.

November 6, 2012

Introduction

Interstitial lung diseases (ILD) affect the tissue (interstitium) and space around the air sacs (alveoli), and differ from obstructive airway diseases. The group includes illnesses caused by inhaled substances, medications, infection, disease, malignancy and unknown causes (idiopathic). Causes of ILD include forms of pulmonary fibrosis and interstitial pneumonia, as well as ILDs associated with connective tissue diseases, exposure to environmental and occupational toxins, malignancy and idiopathic origin (unknown cause). The scarring associated with ILD affects a person’s ability to breathe and, subsequently, oxygenate the bloodstream.

Although scarring is mostly irreversible, medication therapies can slow further progression of lung damage. Pulmonary treatments and oxygen therapy can provide relief to patients. In severe cases, surgery and lung transplants can be an option for some ILD patients.

For More Information

On Cleveland Clinic
Cleveland Clinic’s Interstitial Lung Disease Program is a national leader in diagnostic and therapeutic procedures for ILD, a group of conditions that cause scarring to the lungs and resulting in limited oxygenation. As a high-volume center, Cleveland Clinic has extensive experience caring for the full range of types of ILD, including idiopathic pulmonary fibrosis, sarcoidosis, and connective tissue disease-related ILD, as well as rare types such as increasing pulmonary alveolar proteinosis (PAP) and lymphangioleiomyomatosis (LAM). We offer pulmonary rehabilitation, oxygen therapy and surgical procedures to maintain lung viability.

At Cleveland Clinic's Respiratory Institute, we provide world-class patient care by combining our strengths in clinical expertise, research and education. Serving nearly 90,000 patients annually, the Respiratory Institute’s unsurpassed expertise and experience attracts patients from all over the world who seek a definitive diagnosis and cutting-edge treatment, often for a rare or complex lung disorder. With more than 60 pulmonologists, allergists/immunologists and critical care specialists, the Respiratory Institute diagnoses and treats a wide range of lung, allergy and breathing-related conditions

On Your Health
MyChart®: Your Personal Health Connection, is a secure, online health management tool that connects Cleveland Clinic patients with their personalized health information. All you need is access to a computer. For more information about MyChart®, call toll-free at 866.915.3383 or send an email to: mychartsupport@ccf.org

A remote second opinion may also be requested from Cleveland Clinic through the secure Cleveland Clinic MyConsult® website. To request a remote second opinion, visit eclevelandclinic.org/myConsult


For Appointments

To make an appointment with Dr. Culver  or  Dr. Parambil or any of our specialists in the Respiratory Institute, please call 216.444.6503 or 800.223.2273, ext. 4650. You can also visit us online at www.clevelandclinic.org/respiratory.


About the Speakers

Daniel Culver, D.O. is a staff physician in the Department of Pulmonary, Allergy, and Critical Care Medicine at Cleveland Clinic's main campus. He is board-certified in internal medicine with additional specialty certification in pulmonary medicine and critical care medicine.

Dr. Culver completed his residency in internal medicine, and a fellowship in pulmonary and critical care medicine at Cleveland Clinic. He 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 SouthPointe Hospital in Warrensville Heights, Ohio. Dr. Culver’s clinical interests include sarcoidosis, pulmonary alveolar proteinosis, critical care and interstitial lung diseases. 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.

Joseph Parambil, M.D. is an associate staff member in the Department of Pulmonary, Allergy and Critical Care Medicine. He is also Assistant Professor of Medicine at Cleveland Clinic’s Lerner College of Medicine. Dr. Parambil is board-certified in internal medicine with additional specialty certification in pulmonary medicine and critical care medicine.

Dr. Parambil completed an internal medicine residency at Henry Ford Hospital in Detroit, as well as a fellowship in pulmonary and critical care medicine at Mayo Clinic in Rochester, Minn. He earned his medical degree from Semmelweis Medical University in Budapest, Hungary. Dr. Parambil completed a surgical internship at Frankford Hospital in Philadelphia. Dr. Parambil is a member of several professional societies, including the American College of Physicians, the American Society of Internal Medicine, American College of Chest Physicians, American Thoracic Society and Pulmonary Hypertension Association.


Let’s Chat About Interstitial Lung Disease

Cleveland_Clinic_Host: Welcome to our Online Health Chat with Cleveland Clinic experts Dr. Daniel Culver and Dr. Joseph Parambil. We are thrilled to have them here today to discuss Interstitial Lung Disease. Let’s begin with some of your questions.

ILD and Associated Diseases

Shamimara: Is interstitial lung disease (ILD) associated with lupus? Is it associated with scleroderma?
Dr_Parambil: Yes, ILD is seen in both lupus and scleroderma. In lupus, it tends to be more acute with acute pneumonia and bleeding in the lungs—whereas in scleroderma, it is more chronic with greater fibrosis patterns.

lbb: I have been diagnosed with sarcoidosis via lymph node biopsies near my lungs. Please describe the potential relationship between sarcoidosis and ILD.
Dr_Culver: Sarcoidosis is a specific form of ILD that may lead to lung scarring over time. It may go away on its own, or may simply persist as lung inflammation without much scarring for years. Sometimes other forms of ILD are misdiagnosed as sarcoidosis, so that is one topic you may discuss with your physician. There are a few sarcoidosis patients who develop lung scarring that looks very similar to other forms of ILD (such as idiopathic pulmonary fibrosis [IPF]), but these are the exception rather than the rule. So, the bottom line is that the correct diagnosis relies on a careful assessment of the physical exam, chest CT pattern, pathology and other clues. If there are mixed signals, it may be useful to seek out a large ILD referral center to go over your case.

Shamimara: Can interstitial lung disease cause dysphonia?
Dr_Parambil: Typically, ILD does not cause dysphonia, or voice disorders. However, many different ILDs (IPF and scleroderma related lung disease) can be associated with gastroesophageal reflux disease (GERD), which can cause dysphonia. And sometimes patients are prescribed steroid inhalers and this can also cause dysphonia.

Gram: Do lung nodules usually develop with nonspecific interstitial pneumonia (NSIP)?
Dr_Parambil: No, lung nodules usually do not develop in NSIP. If they develop in a patient with NSIP who is on immunosuppressive medication, we would want to rule out an infection.

Gram: What would cause a high white blood cell count in someone with nonspecific interstitial pneumonia (NSIP)? Is it related to the disease, or something else altogether?
Dr_Parambil: NSIP, regardless of the cause, does not cause a high white blood cell count. The most common reason for this is usually due to treatment with steroids that can raise the white blood cell count. Other immunosuppressants usually do not have that effect. Also, one always has to be mindful that since the immune system is suppressed when NSIP is treated, occult infection can always raise the white blood cell count.

dthor: Can desquamative interstitial pneumonia (DIP) turn into ILD and vice versa?
Dr_Parambil: DIP is, in fact, a type of ILD. Interstitial lung a disease is a broad generic term for all diseases that affect the lung parenchyma, with attention to the interstitial space between the gas-carrying alveoli and the blood-carrying capillaries. Since DIP, a smoking-related ILD, affects this space, it is a type of ILD.

Jsweetie: If your pulmonary function tests are 115 percent and 120 percent, and the technician said normal is 80 percent, does that mean you are in early stages of diagnosed ILD?
Dr_Parambil: Anything higher than 80 percent is considered normal—but it is all relative. If you started off with an FVC (forced vital capacity) of 115 percent two years ago and have now dropped to 80 percent, although it is within normal range it is still a drop, and would warrant further investigation into ILD being a potential explanation for this.

lbb: Have there been links between ILD and common variable immunodeficiency disease (CVID)? One of my doctors has a suspicion that I may have CVID due to the commonplace infections in my eyes and ears. When the infections aren’t treated with antibiotics, these infections typically go into the lungs for me. Just having sarcoidosis doesn't seem to be an excuse for how the infections always transfer to my lungs, so I am wondering if CVID has been linked to ILD—or if eye and ear infections have been linked to ILD.
Dr_Culver: Immune dysregulation syndromes, including CVID, may cause ILD. Sometimes there may be poorly formed granulomas, leading to a misdiagnosis of sarcoidosis. Other times, there may be other microscopic patterns. Tip-offs for this diagnosis include recurring infections and autoimmune diseases. It can usually be diagnosed with blood work, often coordinated by an allergist or immunologist.

Treatment typically includes replacement immunoglobulin , but also must include immunosuppressives, such as steroids, cytotoxics (e.g., azathioprine) or newer agents like rituximab. There are a wide range of immunosuppressives available to choose from.

Jsweetie: Generally speaking, is there more reason to have an open lung biopsy than to not have one when diagnosed with ILD?
Dr_Parambil: This is not an easy question because there are so many variables that go into deciding whether a surgical lung biopsy is needed. But I will say that imaging techniques are getting more refined. In combination with other clinical information, CT scans and specific blood work, a specialist in ILD can accurately predict the type of ILD, and this may even make a lung biopsy unnecessary. However, there are still cases where a surgical lung biopsy is needed to determine the pathologic pattern (disease pathway) of ILD.

Jsweetie: What is a normal DLCO (diffusing capacity for carbon monoxide) score?
Dr_Parambil: Just like most values in pulmonary function tests, the normal predicted value for DLCO is greater than 80 percent, and the actual number is influenced by age, height, weight and sex.

Shamimara: I always have a lot of mucus in my throat. Can that be caused by ILD?
Dr_Culver: Common problems that produce mucus in your throat can include post-nasal drainage (you can test this with a short trial of Sudafed®, if your blood pressure and heart are not problems), acid reflux (this is common in ILD and can cause mucus build-up in the throat, even when there is no heartburn), or recurrent cough from IPF. Some types of ILD may cause very large amount of mucus secretion in the airways. Those situations may require other approaches like anticholinergic inhalers (ipratropium), mucolytics (e.g., Mucinex®) or other therapies.

there4jsweetie: Is it possible to have a pulmonary function test (PFT) that is at 100 percent and oxygen levels that are at 99 percent during a six-minute walk after a diagnosis of pulmonary fibrosis? Does this represent the fact you might have been misdiagnosed?
Dr_Culver: That's possible, but remember that you must accumulate enough disease burden to have an effect on those numbers. So it's possible that the IlD is being seen very early. Also, remember than the PFTs are just population averages. Maybe you were at 120 percent five years ago. In that case, you may have quite a bit of progression. Think about it like IQ. If Albert Einstein saw you and you found he had an IQ of 100, you would not say that the head injury he sustained last week was not having any effect!


Prognosis

nancyp4214: If ILD is caused by scleroderma, can a person live with it for many years before it progresses?
Dr_Parambil: Yes. ILD in the setting of scleroderma can be non-progressive and present for years. We are still slowly figuring out genetic and inflammatory markers that may be associated with who is at risk for progression or not in the presence of ILD .

kdela: Are we any closer to determining the stages and/or progression of this disease? Some days I feel great and think I could go for another 10 years. Other days, I am very lethargic and not nearly optimistic.
Dr_Culver: The progression of the various forms of ILD is quite variable—it depends on the form you are dealing with. However, the progression of ILD is often discussed as it relates to idiopathic pulmonary fibrosis (IPF).

We have learned a good bit about IPF by examining large groups of IPF patients who are enrolled in clinical trials of medications for IPF, and those patients who are assigned to receive placebo. These patients are all evaluated with similar measurements and careful assessments, so we can say that we are comparing apples-to-apples when we discuss them.

In general, IPF can take three courses. About 10 percent of people will have a very fast decline in the first year after the diagnosis. Some of these people will have ‘acute exacerbations’, where their lung function and physical capacity deteriorate suddenly until they reach a new plateau. About 40 percent of people will exhibit good stability over the first year, and will only show very slow declines. And about 50 percent of IPF patients will have a gradual decline.

Having good days and bad days is a common description of symptoms. There may be other factors that contribute to it, such as mucus, airway constriction (like asthma), changes in the weather, or just part of the disease itself.

nancyp4214: Do most people with ILD end up dying from it or from other causes?
Dr_Culver: It depends on the type of ILD. Idiopathic pulmonary fibrosis is usually the cause of death when it is present. Other ILD, like sarcoidosis or connective tissue disease-related ILD, would be less likely, but could possibly be the cause of death. It is also important to remember that therapies or complications of the disease may be the cause of death, not the disease itself.

devo: What is the prognosis for a 74-year-old male diagnosed with silicosis and asbestosis related to working in construction, but retired now?
Dr_Parambil: This is hard to answer, since so much information is absent. But in general, silicosis becomes troublesome to the patient when it leads to progressive massive fibrosis of the upper lobes. Asbestosis on the other hand tends to affect the lower lobes and resembles idiopathic pulmonary fibrosis (IPF), but goes at a much slower pace. So, overall, these fibrotic inhalational lung diseases (ILD) tend to be slowly progressing and not as rapid as idiopathic pulmonary fibrosis.


ILD Medications

nancyp4214: I have ILD caused by scleroderma and started taking CellCept® two months ago. It is causing my white blood cell counts to drop below 2.0. I have been getting Neupogen® shots to boost the counts. If I am unable to continue taking immunosuppressants, is there any other treatment for ILD other than a transplant?
Dr_Culver: Side effects from some of the medications used to treat inflammatory forms of ILD, including scleroderma, may include low blood cell counts from bone marrow suppression. In general, this issue occurs in less than 10 percent of people on these medications, and is the reason why periodic blood work monitoring is necessary. However, having low blood cell counts due to one medication does not necessarily mean that another medication would cause the same issue.

kdela: Is there any medication or procedure to reduce or remove scar tissue in other parts of the body? If so, why can it not be applied to lungs?
Dr_Parambil: Unfortunately, the methods available to remove scar tissue surgically, as would occur in the skin, abdomen or joints, cannot be applied to the lungs. As with any procedure, the surgery leads to added injury and subsequent healing in the proper manner. Unfortunately, if the lungs are injured, this leads to gas exchange issues that can be detrimental to life. With regards to medical management, there is no currently available medicine to dissolve scars.

dmp:What are the side effects of steroids taken for idiopathic pulmonary fibrosis (IPF)?
Dr_Parambil: Current evidence suggests that steroids should not be used to treat IPF. Moreover, the side effects of steroids are not unique to IPF, but are similar when steroids are used to treat a variety of other chronic conditions, including cataracts, acne, weight gain, sleep apnea, high blood pressure, diabetes, osteoporosis, increased risk of infection, and mood abnormalities.

skh727: What is the most widely used medication, for ILD, and how does it affect the kidneys? I have scleroderma with ILD and kidney vasculitis. I was told steroids were not an option.
Dr_Culver: One of the concerns with using steroids in scleroderma is that it may cause a ‘scleroderma renal crisis’, making the kidney problem worse. So, we usually look for other options.

Some of the options may cause kidney problems, but most of them simply require closer monitoring when there is kidney disease, since the level can be higher than in patients with normal kidney function. For your situation, a possible good option that has little risk to the kidneys, but seems to be quite useful for scleroderma, is mycophenolate.


Pulmonary Fibrosis and Acid Reflux

Jsweetie: By treating acid reflux, if there are no other known reasons for pulmonary fibrosis, have there been any cases where scarring has not progressed further?
Dr_Culver: Acid reflux is a hot topic right now, and its role in driving pulmonary fibrosis is still debated. There have been some case series studies describing improvement of pulmonary fibrosis after treatment with acid-blocking medications or even after surgery for acid reflux. Our practice is to treat symptomatic acid reflux right now, and to look hard for it if there are clues suggesting it may be a problem.


Clinical Research Trial Medications

Jsweetie: Can you give us some information on this drug for IPF (idiopathic pulmonary fibrosis) called Vargatef™ (BIBF1120)?
Dr_Parambil: BIBF1120 is a triple kinase inhibitor, which means it blocks three critical growth factors thought to be significant in the progression of IPF. (The molecules that are blocked include VEGF, PDGF and FGF.) A large, randomized study showed that a higher dose of this drug did indeed slow down progression of IPF, diagnosed as a decline in lung function by FVC (forced vital capacity) test . Side effects included gastrointestinal upset.  The FDA is evaluating whether this molecule is ready to be approved for IPF.

want_more: Can you talk about the clinical trials of Pirfenidone®, and how a patient can become involved?
Dr_Culver: Pirfenidone® is an oral drug that has several effects that can lead to blocking of lung scarring (fibrosis), although the exact mechanism of action is still not entirely clear. There have been several trials of Pirfenidone® for idiopathic pulmonary fibrosis (IPF). The results of these trials led to regulatory approval for IPF in Europe, Japan and India. However, the trials did not show consistent results, and the FDA specified that another trial (the ASCEND trial) needed to be performed in the U.S. You can find information about the trial at www.clinicaltrials.gov, which would give you a contact for getting in touch with centers near you. The trial is a little over halfway done, and we are participating in it as well.


Lifestyle Modifications and Supplemental Oxygen

nancyp4214: What determines when supplemental oxygen is needed?
Dr_Culver: Supplemental oxygen is usually given when the oxygen level is low. The level is measured on the finger when the patient is at rest or upon ‘exertion’ (after exercise). We typically like the oxygen level to be stable, which is greater than 92 percent at rest. We may suggest supplemental oxygen with exertion if it drops below 88 percent. It is unclear whether very brief drops into the 85-percent to 90-percent level are important to survival. However, we do know that giving supplemental oxygen improves exercise capacity. Another important use of supplemental oxygen is during the night, when you may spend many hours with low oxygen levels. This would be a significant strain on your heart.

there4jsweetie: Are there any lifestyle, or living environment changes that are recommended to slow down the progression of ILD?
Dr_Parambil: We always recommend an exercise program to our patients with ILD. Leg exercises for conditioning can take away a significant component of the shortness of breath experienced by patients with ILD. With regards to living environment, some ILDs, especially hypersensitivity pneumonitis may be an allergic reaction to antigens in the home, at work or related to certain hobbies. Avoiding the source of these allergens would not only help slow down but may even improve lung function .

lbb: What is your thought on ‘recreational oxygen’ or travel containers for refreshing oxygen upon changing altitudes for those with asthma or another breathing difficulty?
Dr_Parambil: There is no data to support recreational use of supplemental oxygen. However, if you have underlying lung disease and would like to go up in altitude, then getting an altitude simulation study to determine whether you need oxygen for that trip would help. If indeed you do need oxygen, then using this at the altitude would alleviate shortness of breath (dyspnea), simply because the air is indeed thinner at higher altitudes.

lbb: Are there certain geographically-defined climates that are better for an individual suffering with ILD and sarcoidosis?
Dr_Culver: I don't know the answer to that. However, there are data that IPF flare-ups occur more commonly in the winter. So, if you have options to go anywhere, perhaps there is some rationale not to be in the cold or the dark. However, that is a very soft suggestion!

Sarcoidosis may be affected by vitamin D levels, which are hugely influenced by the sun. However, the relationship is very complex, so I do not think we can make a hard-and-fast suggestion about where to live at this time for sarcoidosis.

Jsweetie: Should a person diagnosed with PF get a flu, pneumonia and shingles shot?
Dr_Culver: Yes, to all three!


Closing

I'm sorry to say that our time with Cleveland Clinic experts Daniel Culver, D.O. and Joseph Parambil, M.D. is now over. Thank you Dr. Culver and Dr. Parambil for taking your time to answer questions today about Interstitial Lung Disease.

Dr_Culver: Thanks to all who patiently waited for the chat to start! I hope you found the information you were looking for.

Dr_Parambil: These were some great questions! For more information please visit us online at www.clevelandclinic.org/respiratory.


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