‘Time lost is lung lost’: Early diagnosis critical in pulmonary fibrosis

NASHVILLE, Tenn. — Delayed diagnosis of pulmonary fibrosis is an unfortunate reality for many patients, but experts are seeking to address this problem by closing knowledge gaps and increasing awareness of the disease.

“There are more than 200 causes for pulmonary fibrosis and the diagnostic process is fairly complex in the sense of understanding the demographic and clinical presentation of patients and investigations required,” Tejaswini Kulkarni, MD, MPH, FCCP, associate professor and director of the interstitial lung disease program within the division of pulmonary, allergy and critical care medicine at The University of Alabama at Birmingham, told Healio.

For physicians who may not specialize in ILD, the complicated nature of pulmonary fibrosis, vague symptoms and need for more extensive testing can prove challenging. In an effort to address this problem, the American College of Chest Physicians (CHEST) in collaboration with Three Lakes Foundation produced a white paper — published online and discussed in a special session at CHEST Annual Meeting — authored by both pulmonologists and primary care physicians that identifies factors contributing to delayed diagnosis.

William Lago

Healio spoke with two of the white paper’s steering committee members, Kulkarni and William Lago, MD, family medicine physician at Wooster Family Health Center with the Cleveland Clinic Foundation, to learn more about the white paper.

Healio: Why is early diagnosis so important in pulmonary fibrosis?

Lago: On average, delayed diagnosis can delay treatment as much as 1 to 5 years. Obviously, that in itself is an issue. Granted, pulmonary fibrosis is a disease that is very hard to treat and, for most people, there is not a definite cure. However, we can really slow down the progression of the disease and give the patient a much better quality of life. Additionally, we have medications to treat the disease, lung transplant for those with severe pulmonary fibrosis and ongoing drug trials in which patients can potentially participate. We can only get to those things, though, if we can get patients to treatment earlier.

Kulkarni: In pulmonary fibrosis, there is a perpetual cycle of impaired injury/repair response that ultimately leads to the formation of scar tissue in the lungs. Most of the currently available treatment options slow down the disease progression or reduce this process of fibrosis formation, but we do not have any medications that can cure or reverse the fibrosis that has already ensued in the lungs. Therefore, it is really important to try and reduce disease progression, which does have an impact on the patient’s quality of life as well as survival rates.

Healio: What prompted you and your colleagues to develop this white paper?

Lago: CHEST is working with Three Lakes Foundation, which is an independent foundation that was created by a family who was affected by pulmonary fibrosis. They wanted to see if there was a way to improve earlier diagnosis of pulmonary fibrosis as well as ways to improve treatment, so they approached CHEST and CHEST decided it was worthwhile to see if we could make a difference.

Kulkarni: During the past few years, there have been a few studies providing additional evidence that delays exist in diagnosis of pulmonary fibrosis, be it idiopathic pulmonary fibrosis, autoimmune-ILD, hypersensitivity pneumonitis or other types of pulmonary fibrosis. With this new evidence, it is essential that we identify and put forth concrete measures to try and reduce this delay and thus help improve outcomes among patients with pulmonary fibrosis.

Three Lakes Foundation is primarily focused on raising awareness and patient support for pulmonary fibrosis. They are primarily known for escalating treatment options for pulmonary fibrosis as well as increasing awareness about the disease itself. Through this partnership with CHEST, which is well known for advancing the best patient outcomes through innovative education, clinical research and team-based care, the goal is to try to double up measures to change the trajectory of diagnosis of pulmonary fibrosis and improve the treatment and care for these patients.

Healio: How did you and your colleagues approach development of this paper?

Lago: We had discussions where we asked questions about what we believe delays diagnosis, comparing our own stories with why we think this happens. Then, we developed surveys that were forwarded to PCPs and general pulmonologists to see what they thought about dealing with patients with pulmonary fibrosis. By developing the white paper, we put together our ideas about the problems we identified and then generated some hypotheses as to how we can overcome those problems.

Kulkarni: Once we initially met as a part of the steering committee for the initiative, we decided that the first step was to create a white paper that could outline the results of the surveys that were designed to assess gaps in diagnosing pulmonary fibrosis.

For PCPs, the questions were created to understand the differential diagnosis that they have in mind: What questions do they ask? How do they approach a patient who presents with shortness of breath? What is their diagnostic algorithm? For community pulmonologists, the intent was to understand if they were able to distinguish pulmonary fibrosis or ILD from other chronic lung conditions, such as COPD and asthma, and to understand their approach to coming to a definitive diagnosis on the type of ILD and determining the subsequent treatment processes.

For both groups, there were questions about familiarity with the guidelines for diagnosis and treatment of idiopathic pulmonary fibrosis. PCPs also answered questions based on a patient case scenario — how a typical patient with pulmonary fibrosis may present. Pulmonologists also answered questions about the diagnostic element when evaluating a patient with ILD and how they approach the CT patterns and discussion of lung biopsy.

Healio: What would you say were the main findings of the surveys?

Lago: We found that, among PCPs, the basic knowledge base about pulmonary fibrosis is actually pretty good, but there are often delays in treatment and diagnosis for a number of reasons. Some of them are patient-driven, some are systems-driven and some are physician-driven. When we looked at the general pulmonologists, they were obviously very comfortable with the basic diagnoses and algorithms, but we still found that there were sometimes delays in initiating treatment with antifibrotic medications or referring patients to an ILD clinic to evaluate for potential lung transplant or clinical trial enrollment.

The data showed that reasons for these delays varied significantly, ranging from what questions we may have asked a patient during their visit, to trying medications for other conditions before we did testing, to how we approach patients with such vague symptoms. It was interesting to evaluate the results as a whole to see where some of these roadblocks to more timely diagnosis and treatment occurred.

Kulkarni: The surveys really highlighted some of the gaps in knowledge and clinical behaviors that contribute to these delays in diagnosis. In terms of primary care, we found that physicians do not always include questions that are targeted toward an understanding of pulmonary fibrosis when evaluating a patient with shortness of breath. For example, these include questions regarding exposures, such as household or occupational exposures; presence of symptoms related to autoimmune conditions; family history of ILD; and questions about medications that are associated with ILD.

Another major challenge that we identified is that the symptoms of pulmonary fibrosis overlap with several common conditions, such as cardiac conditions or chronic lung diseases like COPD and asthma. Therefore, only a minority of PCPs actually said they would recommend a more complete workup with spirometry, pulmonary function tests or CT scans, especially if the chest X-ray or radiograph is unremarkable or shows only minor changes. These results really helped inform us about what needs to be included in our toolkit targeted toward PCPs.

When we look at the pulmonologists’ survey results, one notable finding is that they have strong knowledge about the guidelines for IPF diagnosis, but it is the process of diagnosis that really is limited. Also, beyond diagnosis, the reported use of antifibrotic agents was not high. Several pulmonologists did report that they routinely prescribe antifibrotic therapy only after the patient shows disease progression, whereas now, in IPF, we have robust data showing that there is benefit to utilizing antifibrotic therapy irrespective of the stage at which the patient was diagnosed. We have to keep in mind that antifibrotic therapies only slow down disease progression; they will not reverse the fibrosis that has already formed. So, starting the therapies early is of prime importance to improve outcomes in patients with IPF.

Healio: What are the major factors contributing to delayed diagnosis?

Lago: The biggest factor, at least for me as a PCP, is that most patients who have early pulmonary fibrosis have very vague symptoms, such as extremely mild shortness of breath, mild chronic cough, fatigue or problems sleeping. When they present with those types of issues to a PCP’s office, frankly, it is hard to pinpoint what the cause is and that differential diagnosis is mammoth.

Additionally, for many of us in primary care, when you have someone who is first presenting with something like this, the patient doesn’t necessarily want to pursue workup because it’s so early. Even when they do start to get worse, most of us will look for problems requiring immediate attention, such as cardiac issues.

Obviously, patient bias plays a role, but physician bias, insofar as how we treat, makes a difference as well. We found that, for instance, if a patient is already being treated for a lung conditions, such as mild asthma or COPD, and a physician does come to the conclusion that the patient’s new problem is also pulmonary, the physician will likely adjust their current medications before starting to think about treatment of another condition. Many of us are not necessarily familiar with jumping straight to a CT scan or pulmonary function test to look for pulmonary fibrosis. Therefore, there can be delays in treatment just from the way that we approach the disease in general.

Another point that came to light is that the PCPs we surveyed tended to think not much can be done with severe pulmonary fibrosis. We may approach it with the idea that we are not going to really make a difference so we will continue to monitor the patient and see what happens.

From the general pulmonology standpoint, the results showed that a lot of the physicians are hesitant to send their patients on to, for instance, an ILD clinic. Instead, they will try and manage these patients themselves, but they don’t have access to all of the tools that an ILD clinic does.

There is also a systems issue. Within the health care field, all of us are faced with tackling health care costs or dealing with insurance to get tests or referrals approved. Additionally, patients may not want to see another specialist or go through more testing. Not only do some patients have high deductibles, but they may not have transportation; they may not have easy access to a specialist; or they may have to wait months for an appointment. Most of them do not want to go through that for a symptom that was initially pretty mild. However, there are things that we as PCPs cannot do. Sometimes, we have to educate the patient about why they need to see someone else, and that is a challenge in itself.

Kulkarni: The main factors contributing to delayed diagnosis relate to awareness — awareness about pulmonary fibrosis, awareness about the disease process and bringing that up within the differential diagnosis of a patient who presents with shortness of breath or cough. From the time of symptom onset to a pulmonologist evaluation or evaluation at a specialized ILD center, several tests are required, including pulmonary function tests, radiological assessment with chest X-ray and high-resolution CT scans and, based on these results, sometimes surgical lung biopsies and histopathological review for a more definitive diagnosis. Each of these steps can be fraught with delays, and within the algorithm of diagnosis of IPF or other tests for ILD, a missed diagnosis further complicates the process, which increases the time to an accurate diagnosis or more extensive testing.

Healio: Did you identify ways to overcome some of these challenges and hopefully achieve earlier diagnosis?

Lago: As a group, we’re coming up with ideas to try to tackle these challenges. One of the reasons they brought PCPs like me into the mix for CHEST is because the white paper is geared toward primary care. We’re also in the process of developing a full toolkit that will include information about pulmonary fibrosis, a pulmonary fibrosis risk questionnaire that includes questions that, frankly, most of us in primary care don’t have the time to ask patients. There are educational tools to teach us about what we need to check. We also have podcasts and informational talks and we are finding different ways to get this information to PCPs so that it’s in the back of their minds when they’re thinking about their differential diagnosis. We’re trying to help bridge that gap and get those patients to pulmonology a little bit earlier.

Kulkarni: The results from the survey and the white paper have given us additional data on the gaps and delays in diagnosis, so the next goal is to produce a toolkit of resources. The primary audience will be PCPs, but it’s meant to be a useful asset for anyone who is involved in diagnosing ILD. This will all be accessible through the CHEST website and there will be efforts to make sure that it is shared with a variety of audiences.

Healio: Do you have a take-home message for our readers?

Lago: For PCPs, we need to realize that we should keep a diagnosis of pulmonary fibrosis in the back of our minds and know that it is a possibility for patients who are coming in with mild shortness of breath or mild cough. Even if the patient just mentions it as you’re leaving the room, you should at least think about it, stay on top of it and keep it in your differential diagnosis. By having that as part of your toolkit, you will be able to diagnose it much more easily and help those patients in the long run.

I would also add that you can go to CHEST’s website and sign up for notifications and to gain access to the different toolkits that will be available soon.

Kulkarni: Diagnosis and management of pulmonary fibrosis is complex, but a comprehensive management of these patients is of utmost importance because only a few therapies exist in the current landscape for the various ILDs, especially IPF. Although there are efforts being made to develop treatments that not only slow down disease progression but perhaps even cure or aid in the reversal of fibrosis as well as improve quality of life among patients with pulmonary fibrosis, ultimately, early diagnosis will remain the key to improving outcomes among patients with pulmonary fibrosis. To quote the white paper: “Time lost is lung lost.”

References:

• Bridging Specialties: Timely diagnosis for ILD patients. Presented at: CHEST Annual Meeting; Oct. 16-19, 2022; Nashville, Tennessee.
• CHEST. Factors driving delays in idiopathic pulmonary fibrosis. https://www.chestnet.org/GUIDELINES-AND-TOPIC-COLLECTIONS/BRIDGING-SPECIALTIES/TIMELY-DIAGNOSIS-FOR-ILD-PATIENTS. Accessed Oct. 14, 2022.