Selected Podcast
Diagnosis of Idiopathic Pulmonary Fibrosis (IPF)
Idiopathic pulmonary fibrosis (IPF) is one of the common forms of interstitial lung disease (those that affect the tissues between airways); it results in scarring and fibroblasts. Early diagnosis is critical to the management of this dangerous condition. Carla Copeland, MD, explains the challenge of diagnosing IPF due to its generic symptoms, which usually indicate more common breathing issues. She discusses recent innovations in how CT scans are categorized to better diagnose IPF and to help recommend patients for biopsies. Learn why Dr. Copeland works with a multidisciplinary discussion team (MDT) to diagnose this condition accurately and to begin treatment as soon as possible.
Featuring:
Carla Copeland, MD
Carla Copeland, MD Specialties includes Pulmonology.Learn more about Carla Copeland, MD
Disclosure Information:
Release Date: January 13, 2023
Expiration Date: January 12, 2026
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Faculty:
Carla Copeland, MD
Assistant Professor in Pulmonology
Dr. Copeland has no relevant financial relationships with ineligible companies to disclose. There is no commercial support for this activity.
Disclosure Information:
Release Date: January 13, 2023
Expiration Date: January 12, 2026
Planners:
Ronan O’Beirne, EdD, MBA
Director, UAB Continuing Medical Education
Katelyn Hiden
Physician Marketing Manager, UAB Health System
The planners have no relevant financial relationships with ineligible companies to disclose.
Faculty:
Carla Copeland, MD
Assistant Professor in Pulmonology
Dr. Copeland has no relevant financial relationships with ineligible companies to disclose. There is no commercial support for this activity.
Transcription:
Melanie Cole (Host): Idiopathic pulmonary fibrosis is one of the most common interstitial lung diseases and can carry high morbidity and mortality. Early recognition and accurate diagnoses of idiopathic pulmonary fibrosis is pivotal for optimal management and improvement in clinical outcomes.
Welcome to UAB MedCast. I'm Melanie Cole. And joining me is Dr. Carla Copeland. She's a pulmonologist and an assistant professor at UAB Medicine. Dr. Copeland, it's a pleasure to have you with us today. As we get into this topic, can you first start by just telling us about interstitial lung diseases, specifically idiopathic pulmonary fibrosis? Tell us a little bit about how prevalent it is, what you've been seeing in the trends.
Dr Carla Copeland: Yeah. Thank you, Melanie, for having me. As you said, I'm Carla Copeland. And so, interstitial lung diseases in general are a very large category of diseases that affect primarily the lung parenchyma rather than the airways. And then, we kind of further categorize those patients with interstitial lung disease most of the time based on clinical history and then radiographic pattern on imaging. And so, idiopathic pulmonary fibrosis is one of the most common interstitial lung diseases that we encounter clinically. And it is what we consider a scarring lung disease or fibrotic lung disease. And it most commonly occurs in older patients, so typically above age 60, and is more common in males than in female.
And then, some of the risk factors that we see for these patients, we're not entirely sure what causes idiopathic pulmonary fibrosis. There's a lot of research going into that, but it does seem to involve an abnormal response to injury in the lungs that leads to scar production and collagen deposition and proliferation of fibrotic fibroblast in the lungs. And the risk factors for this lung process can include things as simple as smoking and acid reflux, but also can include viral infections and other lung injury as well as family history.
Melanie Cole (Host): Dr. Copeland, why is it sometimes challenging to diagnose? Why is early recognition and accurate diagnosis pivotal for those optimal management? Tell us a little bit about diagnosis and what you're looking for.
Dr Carla Copeland: Certainly, yeah. So, I think what is challenging about this disease is that the symptoms that patients present with are so ubiquitous. So, most of these patients present with shortness of breath and cough, and that can come from so many different places that, really, there needs to be a high index of suspicion for these patients. A lot of times because it is a rare disease, people don't think about it as commonly as I think about things like COPD or asthma or some of these other pulmonary diseases. So, I think really a lot of times in primary care clinics, it's further down the algorithm of what people think about in diagnosis. And so, it sometimes takes some time and then by the time patients get imaging that shows, "Oh, hey, they have lung scarring." Then, it's a lot of times a little bit further into the disease course. And so, I think that's why it's so difficult to diagnose and why we sometimes see delays in recognition.
Now, in terms of kind of the second portion of your question as to how do we diagnose this, so we have clinical guidelines that we go off of and the mainstay of how we diagnose idiopathic pulmonary fibrosis or IPF is with high-resolution computed tomography or CT imaging. And so, this is a specialized CT that uses very thin slices to get very high resolution images. And then, we take a picture when patients take a deep breath in, a picture when they take a deep breath out. And that kind of helps us characterize a lung disease.
And really, the way we look at the images once they're done is we try to categorize them into a pattern that we know is associated most commonly with idiopathic pulmonary fibrosis. And so, there's a CT pattern called usual interstitial pneumonia or UIP. And that pattern is what we look for when we're concerned about a diagnosis of idiopathic pulmonary fibrosis. And really, the defining feature of this on a CT imaging is going to be presence of honeycombing, which is these little cysts that sit in little rows, kind of right under the pleural space, as well as other fibrotic features, which include abnormal dilation of the airways in the fibrotic areas, which we call traction bronchiectasis; and then also reticulations, which are these linear areas of fibrosis that come off of the subpleural space or that area right below the lining of the lung. And so, really that's the pattern we're looking for in diagnosing these patients. And so, really in order to diagnose it, physicians, general pulmonologists, as well as primary care providers have to think about getting that type of image. And sometimes it's a little bit further down the diagnostic process.
Melanie Cole (Host): That's so interesting. And Dr. Copeland, as I understand it, those clinical practice guidelines you mentioned were updated recently. What's different now? What has changed? As you spoke about the imaging and you spoke about that process, what's changed?
Dr Carla Copeland: So, the original guidelines came out in 2018 that helped define this imaging pattern, this usual interstitial pneumonia pattern. And within those guidelines, there was kind of four categories that we tried to put these CTs into, so the commonly associated usual interstitial pneumonia pattern. And then, there's another pattern called probable usual interstitial pneumonia and indeterminate and alternative diagnosis. So, alternative diagnosis means the imaging findings don't really fit with the classic pattern we see in idiopathic pulmonary fibrosis or that UIP pattern.
And so, really the biggest change in terms of the imaging diagnosis was the usual interstitial pneumonia pattern. And then, the probable usual interstitial pneumonia pattern were grouped together similarly in diagnostic algorithm. So really, kind of in general, the diagnostic algorithm is that we look at the imaging. And if it fits the usual interstitial pneumonia pattern, then we make sure no other processes are driving this pattern on imaging. So, sometimes connective tissue diseases can cause this pattern. So, we send serologies or blood tests to look for those. And then, sometimes environmental exposures can lead to this pattern. And so, we ask a very detailed history about things that patients might get exposed to, both occupationally and domestically.
And if we go through that algorithm and don't find any other causes for the usual interstitial pneumonia pattern, then we can give it a confident diagnosis of IPF or idiopathic pulmonary fibrosis. And then, in the prior guidelines, the probable usual interstitial pneumonia pattern, which by definition has some fibrotic features, so reticulation or those linear structures coming off of the subpleural space and traction bronchiectasis or kind of abnormal dilation of the airways. So, if that pattern was present in the last iteration of the guidelines in 2018, it wasn't as diagnostic of IPF. But now, we know that we have more information and more data saying that the probable UIP pattern tends to progress similarly to the UIP pattern and has a pretty high positive predictive value for coming up with the usual interstitial pneumonia pattern on biopsy, which is the gold standard. And so, we've found that seeing this imaging pattern, really, you go down the same diagnostic algorithm now. So, the UIP and probable UIP have the same clinical diagnostic algorithm now. So, I think that's probably the biggest change in terms of diagnosis.
And then, the other big change would be the types of biopsies that we can do. And so, the recommendation is if you have a UIP or probable UIP pattern, you don't really need to biopsy these patients. But some of these patterns that don't look as classic for idiopathic pulmonary fibrosis sometimes may need biopsies. And classically, that's been surgical lung biopsies, which you can imagine are large procedures that require hospital stays. And so, people thought about other ways that we could biopsy these patients that maybe were a little less invasive. And so, there is what is called a transbronchial lung cryobiopsy now, which is essentially a bronchoscopic procedure that takes a section of long via frozen biopsy procedure. And so, the new guidelines have allowed transbronchial cryobiopsy to be an acceptable alternative in the diagnosis of some of those patients who don't fit the classic pattern, and that we're concerned about idiopathic pulmonary fibrosis or potentially other interstitial lung diseases.
So, I think those are the two biggest updates in diagnosis, would be the addition of transbronchial cryobiopsy, as well as grouping probable UIP pattern into the diagnostic algorithm with UIP.
Melanie Cole (Host): Well, thank you for that, and I'm glad that you brought up the detailed history, whether it's environmental exposures, potential causes that really is so helpful to hear for other healthcare providers. As we get ready to wrap up, tell us a little bit about what's next in diagnosis. Once it happens, once you have determined this differential diagnosis, what happens then? A multidisciplinary team gets involved. What next?
Dr Carla Copeland: Yes. Multidisciplinary discussion or what's called MDD for short is considered the gold standard for diagnosing interstitial lung disease. And I think that's because it is such a nuanced and sometimes complicated diagnosis to make. And so, the multidisciplinary discussion team includes pulmonologists, typically those who are experts in the interstitial lung disease sector of pulmonology as well as radiologists, typically thoracically-trained radiologists, and then pathologists, ideally those who are thoracically-trained. And it is an interactive discussion of cases between all of these members, giving both the clinical history, the radiographic pattern. And then, if a biopsy is performed, discussing the biopsy results. And so, that is considered certainly the gold standard and typically how we diagnose some of these more complicated patients. And a lot of times after we have imaging and clinical history, we sit down and talk about these patients. And that is still in the guidelines, the recommendation for diagnosis.
Melanie Cole (Host): And lastly, what would you like other providers to take away from this podcast on diagnosis of interstitial lung disease and specifically idiopathic pulmonary fibrosis and when you feel it's important they refer to the specialists at UAB Medicine?
Dr Carla Copeland: I think really early recognition is key and having a high index of suspicion in these patients. So if you have an older patient, 60 year old above, and they're having shortness of brass and cough, maybe they have some risk factors, think about idiopathic pulmonary fibrosis because we can't diagnose it unless somebody recognizes it or thinks about it along the way. So, I think that's the biggest thing, is just to think about it as you go along. And then, certainly, if you see imaging findings that are concerning for fibrotic changes, please refer to a pulmonary provider. And then, if that pulmonary provider needs to, they can refer to our interstitial lung disease team as well. But certainly, they should see a pulmonologist to determine next best steps. So, I think that would be the biggest takeaway. And then, starting to get used to those patterns on imaging. Certainly for the pulmonologist, they should certainly be able to recognize the usual interstitial pneumonia pattern. Maybe not the primary care providers, but they should at least start to get comfortable with the language we use there.
Melanie Cole (Host): Thank you so much, Dr. Copeland. That was so educational and informative. Thank you again. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST or by visiting our website at uabmedicine.org/physician. That concludes this episode of UAB MedCast. I'm Melanie Cole.
Melanie Cole (Host): Idiopathic pulmonary fibrosis is one of the most common interstitial lung diseases and can carry high morbidity and mortality. Early recognition and accurate diagnoses of idiopathic pulmonary fibrosis is pivotal for optimal management and improvement in clinical outcomes.
Welcome to UAB MedCast. I'm Melanie Cole. And joining me is Dr. Carla Copeland. She's a pulmonologist and an assistant professor at UAB Medicine. Dr. Copeland, it's a pleasure to have you with us today. As we get into this topic, can you first start by just telling us about interstitial lung diseases, specifically idiopathic pulmonary fibrosis? Tell us a little bit about how prevalent it is, what you've been seeing in the trends.
Dr Carla Copeland: Yeah. Thank you, Melanie, for having me. As you said, I'm Carla Copeland. And so, interstitial lung diseases in general are a very large category of diseases that affect primarily the lung parenchyma rather than the airways. And then, we kind of further categorize those patients with interstitial lung disease most of the time based on clinical history and then radiographic pattern on imaging. And so, idiopathic pulmonary fibrosis is one of the most common interstitial lung diseases that we encounter clinically. And it is what we consider a scarring lung disease or fibrotic lung disease. And it most commonly occurs in older patients, so typically above age 60, and is more common in males than in female.
And then, some of the risk factors that we see for these patients, we're not entirely sure what causes idiopathic pulmonary fibrosis. There's a lot of research going into that, but it does seem to involve an abnormal response to injury in the lungs that leads to scar production and collagen deposition and proliferation of fibrotic fibroblast in the lungs. And the risk factors for this lung process can include things as simple as smoking and acid reflux, but also can include viral infections and other lung injury as well as family history.
Melanie Cole (Host): Dr. Copeland, why is it sometimes challenging to diagnose? Why is early recognition and accurate diagnosis pivotal for those optimal management? Tell us a little bit about diagnosis and what you're looking for.
Dr Carla Copeland: Certainly, yeah. So, I think what is challenging about this disease is that the symptoms that patients present with are so ubiquitous. So, most of these patients present with shortness of breath and cough, and that can come from so many different places that, really, there needs to be a high index of suspicion for these patients. A lot of times because it is a rare disease, people don't think about it as commonly as I think about things like COPD or asthma or some of these other pulmonary diseases. So, I think really a lot of times in primary care clinics, it's further down the algorithm of what people think about in diagnosis. And so, it sometimes takes some time and then by the time patients get imaging that shows, "Oh, hey, they have lung scarring." Then, it's a lot of times a little bit further into the disease course. And so, I think that's why it's so difficult to diagnose and why we sometimes see delays in recognition.
Now, in terms of kind of the second portion of your question as to how do we diagnose this, so we have clinical guidelines that we go off of and the mainstay of how we diagnose idiopathic pulmonary fibrosis or IPF is with high-resolution computed tomography or CT imaging. And so, this is a specialized CT that uses very thin slices to get very high resolution images. And then, we take a picture when patients take a deep breath in, a picture when they take a deep breath out. And that kind of helps us characterize a lung disease.
And really, the way we look at the images once they're done is we try to categorize them into a pattern that we know is associated most commonly with idiopathic pulmonary fibrosis. And so, there's a CT pattern called usual interstitial pneumonia or UIP. And that pattern is what we look for when we're concerned about a diagnosis of idiopathic pulmonary fibrosis. And really, the defining feature of this on a CT imaging is going to be presence of honeycombing, which is these little cysts that sit in little rows, kind of right under the pleural space, as well as other fibrotic features, which include abnormal dilation of the airways in the fibrotic areas, which we call traction bronchiectasis; and then also reticulations, which are these linear areas of fibrosis that come off of the subpleural space or that area right below the lining of the lung. And so, really that's the pattern we're looking for in diagnosing these patients. And so, really in order to diagnose it, physicians, general pulmonologists, as well as primary care providers have to think about getting that type of image. And sometimes it's a little bit further down the diagnostic process.
Melanie Cole (Host): That's so interesting. And Dr. Copeland, as I understand it, those clinical practice guidelines you mentioned were updated recently. What's different now? What has changed? As you spoke about the imaging and you spoke about that process, what's changed?
Dr Carla Copeland: So, the original guidelines came out in 2018 that helped define this imaging pattern, this usual interstitial pneumonia pattern. And within those guidelines, there was kind of four categories that we tried to put these CTs into, so the commonly associated usual interstitial pneumonia pattern. And then, there's another pattern called probable usual interstitial pneumonia and indeterminate and alternative diagnosis. So, alternative diagnosis means the imaging findings don't really fit with the classic pattern we see in idiopathic pulmonary fibrosis or that UIP pattern.
And so, really the biggest change in terms of the imaging diagnosis was the usual interstitial pneumonia pattern. And then, the probable usual interstitial pneumonia pattern were grouped together similarly in diagnostic algorithm. So really, kind of in general, the diagnostic algorithm is that we look at the imaging. And if it fits the usual interstitial pneumonia pattern, then we make sure no other processes are driving this pattern on imaging. So, sometimes connective tissue diseases can cause this pattern. So, we send serologies or blood tests to look for those. And then, sometimes environmental exposures can lead to this pattern. And so, we ask a very detailed history about things that patients might get exposed to, both occupationally and domestically.
And if we go through that algorithm and don't find any other causes for the usual interstitial pneumonia pattern, then we can give it a confident diagnosis of IPF or idiopathic pulmonary fibrosis. And then, in the prior guidelines, the probable usual interstitial pneumonia pattern, which by definition has some fibrotic features, so reticulation or those linear structures coming off of the subpleural space and traction bronchiectasis or kind of abnormal dilation of the airways. So, if that pattern was present in the last iteration of the guidelines in 2018, it wasn't as diagnostic of IPF. But now, we know that we have more information and more data saying that the probable UIP pattern tends to progress similarly to the UIP pattern and has a pretty high positive predictive value for coming up with the usual interstitial pneumonia pattern on biopsy, which is the gold standard. And so, we've found that seeing this imaging pattern, really, you go down the same diagnostic algorithm now. So, the UIP and probable UIP have the same clinical diagnostic algorithm now. So, I think that's probably the biggest change in terms of diagnosis.
And then, the other big change would be the types of biopsies that we can do. And so, the recommendation is if you have a UIP or probable UIP pattern, you don't really need to biopsy these patients. But some of these patterns that don't look as classic for idiopathic pulmonary fibrosis sometimes may need biopsies. And classically, that's been surgical lung biopsies, which you can imagine are large procedures that require hospital stays. And so, people thought about other ways that we could biopsy these patients that maybe were a little less invasive. And so, there is what is called a transbronchial lung cryobiopsy now, which is essentially a bronchoscopic procedure that takes a section of long via frozen biopsy procedure. And so, the new guidelines have allowed transbronchial cryobiopsy to be an acceptable alternative in the diagnosis of some of those patients who don't fit the classic pattern, and that we're concerned about idiopathic pulmonary fibrosis or potentially other interstitial lung diseases.
So, I think those are the two biggest updates in diagnosis, would be the addition of transbronchial cryobiopsy, as well as grouping probable UIP pattern into the diagnostic algorithm with UIP.
Melanie Cole (Host): Well, thank you for that, and I'm glad that you brought up the detailed history, whether it's environmental exposures, potential causes that really is so helpful to hear for other healthcare providers. As we get ready to wrap up, tell us a little bit about what's next in diagnosis. Once it happens, once you have determined this differential diagnosis, what happens then? A multidisciplinary team gets involved. What next?
Dr Carla Copeland: Yes. Multidisciplinary discussion or what's called MDD for short is considered the gold standard for diagnosing interstitial lung disease. And I think that's because it is such a nuanced and sometimes complicated diagnosis to make. And so, the multidisciplinary discussion team includes pulmonologists, typically those who are experts in the interstitial lung disease sector of pulmonology as well as radiologists, typically thoracically-trained radiologists, and then pathologists, ideally those who are thoracically-trained. And it is an interactive discussion of cases between all of these members, giving both the clinical history, the radiographic pattern. And then, if a biopsy is performed, discussing the biopsy results. And so, that is considered certainly the gold standard and typically how we diagnose some of these more complicated patients. And a lot of times after we have imaging and clinical history, we sit down and talk about these patients. And that is still in the guidelines, the recommendation for diagnosis.
Melanie Cole (Host): And lastly, what would you like other providers to take away from this podcast on diagnosis of interstitial lung disease and specifically idiopathic pulmonary fibrosis and when you feel it's important they refer to the specialists at UAB Medicine?
Dr Carla Copeland: I think really early recognition is key and having a high index of suspicion in these patients. So if you have an older patient, 60 year old above, and they're having shortness of brass and cough, maybe they have some risk factors, think about idiopathic pulmonary fibrosis because we can't diagnose it unless somebody recognizes it or thinks about it along the way. So, I think that's the biggest thing, is just to think about it as you go along. And then, certainly, if you see imaging findings that are concerning for fibrotic changes, please refer to a pulmonary provider. And then, if that pulmonary provider needs to, they can refer to our interstitial lung disease team as well. But certainly, they should see a pulmonologist to determine next best steps. So, I think that would be the biggest takeaway. And then, starting to get used to those patterns on imaging. Certainly for the pulmonologist, they should certainly be able to recognize the usual interstitial pneumonia pattern. Maybe not the primary care providers, but they should at least start to get comfortable with the language we use there.
Melanie Cole (Host): Thank you so much, Dr. Copeland. That was so educational and informative. Thank you again. And a physician can refer a patient to UAB Medicine by calling the MIST line at 1-800-UAB-MIST or by visiting our website at uabmedicine.org/physician. That concludes this episode of UAB MedCast. I'm Melanie Cole.