A steering committee of pulmonary experts and primary care physicians (PCPs) released today a white paper outlining the issues surrounding delays in diagnosing interstitial lung diseases (ILDs) like pulmonary fibrosis (PF). Brought together by the American College of Chest Physicians (CHEST) and Three Lakes Foundation, the committee provides expert guidance for the initiative, Bridging Specialties™: Timely Diagnosis for ILD Patients, with the goal of reducing the time it takes to reach a diagnosis for complex lung diseases.
The clinical perspective is based on data collected from surveys sent to PCPs and pulmonologists to assess the need for interventions, including tools that can be used to aid in diagnosing PF and other ILDs.
As it relates to the primary care clinician, the objective of the survey was to:
- Assess behaviors of primary care physicians in response to a patient presenting with nonspecific symptoms
- Measure knowledge and attitudes regarding a diagnosis of PF or idiopathic pulmonary fibrosis (IPF)
For the pulmonary specialist, the objective of the survey was to identify what distinguishes ILDs from other, more common, lung issues.
Data were analyzed by specialty, familiarity with PF guidelines, experience diagnosing patients with PF, region of the United States, type of area served by their practice (both urban/rural/suburban and socioeconomic status of the community served) and tenure in practice.
Based on the results of the surveys:
- PCPs and pulmonologists agree-;there is no short list of factors that contribute to delays in diagnosis of IPF. Overcoming barriers is a complex challenge because the issues driving delays are multifactorial.
- Patients presenting with nonspecific symptoms that may be early warning signs are much more likely to be evaluated for cardiac conditions (#1 differential diagnosis for 40% of PCP respondents), followed by COPD and asthma. ILD/IPF is not a top-of-mind consideration-;less than half of respondents (42%) considered it as one of their top three diagnoses for a patient presenting as such.
- While, in general, 87% of PCPs will try to evaluate the root causes of nonspecific symptoms, that number drops to 61% if the patient is already on inhaled therapy for a pulmonary condition. This means that a substantial minority of PCPs (39%) will bypass symptom evaluation to modulate therapy for what may be an incorrect diagnosis.
- High-resolution computed tomography (HRCT)-;the gold standard of imaging as it relates to detecting ILD-;is not universally ordered for patients when initial diagnostics justify that step. Only 62% say they order HRCT when a patient's chest radiograph shows lower lobe opacity, and only 50% say they order it when a patient has inspiratory crackles or some other abnormal pulmonary exam.
"As a practicing primary care physician, it doesn't surprise me that PF/IPF are generally misdiagnosed or experience delays in diagnosis. These diseases are on the rarer side, so when a patient comes to their PCP, that doctor first will opt to rule out heart issues that can quickly end a life," says steering committee member and family medicine physician, William Lago, MD. "That said, lung diseases like PF are incredibly difficult to live with and can progress rapidly if untreated. An earlier diagnosis means starting treatments to slow fibrosing of the lungs and, with slowed disease progression, a patient's quality of life is often improved."
The results of the surveys show that there are opportunities to close knowledge gaps that will elevate ILD and PF as a differential diagnosis in the early stages of symptom presentation and speed access to appropriate referral, especially as it relates to awareness of early symptomatic clues and common comorbidities. Guided by these findings, the steering committee will produce resources as part of a tool kit to more quickly recognize these complex lung diseases.
As someone who works daily with interstitial lung disease, it is common for me to encounter what is an incredibly rare disease for other clinicians. Based on these survey results, and from what we are hearing in shared patient experiences, increasing awareness about this disease and providing additional training in the diagnostic algorithm is of prime importance. Close collaboration between primary care and pulmonary medicine will define what the resources will be to ultimately shorten the time to diagnosis."
Tejaswini Kulkarni, MD, MPH, FCCP, steering committee member and pulmonologist
To learn more about the Bridging Specialties initiative, to download the full white paper and to sign up for updates as resources become available, visit https://www.chestnet.org/Guidelines-and-Topic-Collections/Bridging-Specialties/Timely-Diagnosis-for-ILD-Patients.