PFF Insights

Improving the pathway to diagnosis and treatment of ILD

by Joyce Lee, MD, Senior Medical Advisor, Research and Health Care Quality
December 29, 2023

People with pulmonary fibrosis (PF) and interstitial lung disease (ILD) often face a long and tough journey before getting a definite diagnosis. This complex path is challenging for both patients and their healthcare providers. Quickly and accurately identifying these conditions is crucial for starting the best management for their condition.

A recent study in the BMJ medical journal looked at how to expedite the diagnostic process. "Defining the pathway to timely diagnosis and treatment of interstitial lung disease: a US Delphi survey," involved primary care doctors and lung specialists. It highlighted symptoms needing attention, steps for diagnosis, and teamwork between medical fields for ILD patients.

It is recognized that symptoms of ILD are frequently insidious and non-specific. Patients often minimize their symptoms or simply attribute them to getting older. When they finally see their primary doctor, they may get a battery of tests for heart and lung problems before being sent to a pulmonologist. Some people even had to see their regular doctor more than four times before getting sent to the right specialist.[1]

This study used Delphi methodology to gain consensus on key areas of focus to reduce diagnostic delays for patients with ILD, including pragmatic considerations for primary care physicians in evaluating patients with respiratory symptoms. It outlines specific symptoms and clinical features that should raise the suspicion for ILD, and the need for increased education and specific guidelines to help ensure timely referral to a pulmonologist. Establishing a consensus in this domain could serve as a blueprint for primary care physicians who encounter ILD infrequently in their practice.

Further, this study identified areas of consensus on the initial work-up for patients with suspected ILD and the type of imaging that is needed, along with suggestions for standard terminology in radiology reports to improve communication. Given that ILD are considered rare conditions, this study also provided consensus statements on the role of multidisciplinary discussion and when referral to a specialist center is recommended to assist with diagnosis and management of these complex diseases.

This research outlines key symptoms and features signaling a need for suspecting ILD in the differential diagnosis and outlines consensus statements to facilitate timely diagnosis in primary care and outpatient pulmonary settings. It emphasizes the importance of smooth transitions between medical specialties for ILD patients and stresses the collaboration required among primary care providers, rheumatologists, community pulmonologists, and ILD specialists to ensure proper care. Enhanced awareness and understanding of the necessary steps can minimize delays in referrals and streamline the process for diagnosing and managing ILD.

[1] Cosgrove  GP, Bianchi  P, Danese  S, et al. Barriers to timely diagnosis of interstitial lung disease in the real world: the INTENSITY survey. BMC Pulm Med 2018;18:9. doi:10.1186/s12890-017-0560-x