A new toolkit aims to help clinicians shorten the time to diagnosis for pulmonary fibrosis and other interstitial lung diseases (ILDs).

The resource, shared on Rare Disease Day (Feb. 28), was developed as part of Bridging Specialties: Timely Diagnosis for ILD, a collaborative initiative by the American College of Chest Physicians (CHEST) and Three Lakes Foundation. Affecting 400,000 people in the US, ILDs are often overlooked as a potential diagnosis given their rarity, according to a press release by CHEST.

A proper diagnosis for this disease is further complicated by ubiquitous presenting symptoms that are common in many other diseases, including asthma, COPD, and cardiac conditions, and often leads to a misdiagnosis. This delay in diagnosis, or an outright misdiagnosis, leads to additional delays in receiving proper treatment and, subsequently, a degradation in the patient’s quality of life.

The toolkit includes: 

  • Patient questionnaire: An ILD-specific patient questionnaire focusing on past and current medications, surgeries, occupational and environmental exposures, and known comorbidities.
  • Decision-making module: An interactive module looking at three patients presenting with differing signs and symptoms that could be indicative of ILDs. The module includes actions to take, including completing a physical exam, ordering testing, and making the determination to provide treatment or refer to a specialist.
  • e-Learning modules: e-Learning modules on symptoms of ILD, including the sound of crackles on auscultation that is commonly heard at the bottom of the lungs in patients with these rare diseases.
  • Expert radiology review videos: Videos exploring radiologic features of ILDs that provide clues of what a clinician should look for when reviewing a radiograph. The videos cover key patterns, common CT appearances, and imaging features that can help in diagnosis.

“Rare Disease Day is a great time to bring awareness to this initiative and the existing gaps in diagnosis of interstitial lung diseases,” says Tejaswini Kulkarni, MD, FCCP, pulmonologist and member of the Bridging Specialties steering committee, in a press release. “By bringing together primary care and pulmonary [medicine], we hope to expose a lot more clinicians, and ultimately patients, to the severity of this complex lung disease.”

To assist in outlining the issue of delays in diagnosis, the Bridging Specialties steering committee also created a video speaking to the initiative and how the toolkit and a strategic partnership between primary care and pulmonary medicine will help to improve care and quality of life for patients who may have interstitial lung disease. 

“In working on this initiative with my pulmonary colleagues, I’m already finding myself thinking more about pulmonary fibrosis and other interstitial lung diseases as potential diagnoses when seeing patients,” says William Lago, MD, primary care physician and member of the Bridging Specialties steering committee, in a press release. “Between the patient questionnaire, the decision-making module, and the other resources in the clinician toolkit, I can see this having an incredible impact on how we diagnose patients.”

Photo credit: The American College of Chest Physicians