Prompt diagnosis of idiopathic pulmonary fibrosis is essential to reduce mortality, and improving education of primary care providers can help, suggests a new white paper.

The nonspecific nature of the symptoms of idiopathic pulmonary fibrosis (IPF) especially in early stages, and the relative rarity of IPF compared with other conditions that have similar symptoms, may contribute to a delay in diagnosis in the primary care setting, wrote Daniel F. Dilling, MD, of Loyola University Chicago, Maywood, Ill., and colleagues in Chest: Clinical Perspectives (Dilling et al. State of Practice: Factors Driving Diagnostic Delays in Idiopathic Pulmonary Fibrosis. Chest. 2022).

“We have learned over and over again through research, and also through talking with our own patients with IPF, that there is often a long lag between the first signs of the disease and a diagnosis of IPF,” corresponding author Dr. Dilling said in an interview.

“Even some pulmonary specialists can be uncertain about how to approach the diagnosis when a CT scan or other test first suggests the possibility; this can cost a patient precious time, as being on drug therapy earlier can result in preservation of lung function,” he said. “By sounding the alarm bell with this paper, we hope to promote awareness and education/training within the primary care community as well as the pulmonary community, and also to make all of them aware of the possibility of referral to specialty ILD [interstitial lung disease] centers when desired and possible,” he added.  

The researchers conducted a pair of online surveys to inform the development of improving education on IPF among primary care providers.

In the white paper, which can be accessed online, the authors reported results of the surveys. One included 100 general pulmonologists and the other included 306 primary care physicians (156 practiced family physicians and 150 practiced general internal medicine). The data were collected between April 11, 2022, and May 16, 2022. Participants were asked to respond to a patient case scenario of a 55-year-old woman with nonspecific symptoms such as shortness of breath on moderate exertion, cough, exhaustion, and trouble sleeping.

The PCPs were most likely to evaluate the patient for a cardiac condition (46%), 25% would evaluate for chronic obstructive pulmonary disease (COPD), and 23% for asthma. More than half (58%) ranked progressive fibrosing ILD as one of their bottom two diagnoses.

A total of 87% of PCPs said they would begin a diagnostic workup to evaluate symptoms if the patient had no preexisting respiratory disease, compared with 61% for patients with a respiratory diagnosis.

Although 93% of PCPs cited a chest x-ray as part of the initial patient workup, fewer than half said they would order an echocardiogram, spirometry, or pulmonary function test (PFT), and 11% said they would include diffusion capacity testing in the initial workup.

In addition, PCPs were less likely to ask patients about issues that might prompt an IPF diagnosis, such as exposures to agents through work, hobbies, the environment, or comorbidities.

In the pulmonology survey, more than 75% of respondents cited patient history, high-resolution tomography scan, serologic testing, and review for autoimmune disease symptoms as first steps in a diagnostic response to patients with suspected IPF.

Differences between PCPs’ and pulmonolgists’ responses

Both PCPs and pulmonologists responded to several questions to assess knowledge and opinion gaps related to IPF. Overall, pulmonologists were more likely than PCPs to cite both imaging and testing issues and waiting 6-8 weeks after symptom onset before imaging as contributing factors to diagnostic delays.

PCPs more often expressed beliefs that delayed diagnosis had little impact on a patient with IPF, and that the treatments may be worse than the disease.

Dr. Dilling said he was not surprised by the survey findings, as similar clues about the underdiagnosis of IPF have surfaced in prior studies.

“We need to get the word out to primary care physicians, to pulmonary physicians, and even to the public, that idiopathic pulmonary fibrosis and other forms of interstitial lung disease are out there and prevalent, and that making the right diagnosis in a timely way can lead to better outcomes for patients,” he said.

The take-home message for primary care is to think outside the COPD box, said Dr. Dilling. “Just because someone has shortness of breath or cough and used to smoke does not automatically mean that they have COPD,” he emphasized. “Listen carefully for crackles (rales) on exam. Get spirometry or PFTs before you secure the diagnosis of COPD, or else you will be missing all of your cases of pulmonary fibrosis; think of pulmonary fibrosis and use imaging to help guide your diagnosis,” he said.

The authors suggested several education goals for PCPs, including establishing the importance of early evaluation, outlining the correct approach to a patient workup, encouraging prompt referral, and empowering PCPs as part of the team approach to IPF patients’ care. For pulmonologists, only 11% of those surveyed said they were aware of the latest developments in antifibrotic research, and education efforts might include information about drug pipelines and clinical trials, as well as technology.

Looking ahead, “We need to better understand how to find the pulmonary fibrosis in the community,” Dr. Dilling said. This understanding may come in part from greater education and awareness, he noted. However, eventually there may be ways to enhance the reading of PFTs and of CT scans through artificial intelligence technologies that would not only prompt clinicians to recognize what they are seeing, but would prompt them to refer and send the patient on the correct diagnostic path as soon as possible, he added.

Key message: Include ILD in differential diagnosis of patients with shortness of breath and/or cough

Advances in diagnostics and therapies for interstitial lung disease can take time to be absorbed and adopted, and patients with ILD and pulmonologists caring for ILD, specifically IPF, continue to report delays in diagnosis and therapy, said Krishna Thavarajah, MD, a pulmonologist at Henry Ford Hospital, Detroit, Mich., in an interview.

The current study findings of the time to diagnosis and the approach to patient workups echo her own clinical experience, Dr. Thavarajah said. “There is a delay in IPF diagnosis as physicians look to more common diagnoses, such as cardiac disease or chronic obstructive pulmonary disease, prior to pursuit of additional workup, and the attitude toward treatment has, in some ways, lagged behind advances in therapy, including timing and feasibility of therapy for IPF,” she said.

The key message for primary care physicians is to include ILD in the differential diagnosis of patients with shortness of breath and/or cough, especially if the initial cardiac and pulmonary test (meaning at least a chest x-ray and pulmonary function tests, including a diffusion capacity) are not pointing to an alternative cause within 3 months of presentation, Dr. Thavarajah said.

Once IPF is diagnosed, primary care clinicians should know that there are FDA-approved therapies that improve survival, said Dr. Thavarajah. “There are identifiable and treatable comorbid conditions,” she added. “The statement of ‘time lost is lung lost’ sums up the care of an IPF patient; partnerships between primary care clinicians, pulmonologists, and referral centers can provide the patient multiple levels of support with quality-of-life interventions, treatments, and also clinical trials, delivered by a team of providers,” she said. 
In the wake of the current study, more research is needed with outcome studies regarding educational interventions targeting primary care and pulmonologists on appropriate workup, timing of workup, and current therapyfor IPF patients, she added.

The white paper received no outside funding. The authors and Dr. Thavarajah had no financial conflicts to disclose.