New Toolkit Targeting Delayed ILD Diagnosis Is Released by Inter-Specialty Group

Interstitial lung disease (ILD) affects an estimated 400,000 people in the US.¹ Because of this, the odds of a primary care physicians encountering a patient with ILD is rare, and symptoms are easily overlooked by clinicians and patients alike. This results in delays — in diagnosis, specialist referrals, and access to treatment.^1,2

A 2021 study found the time to ILD diagnosis was greater than 1 year in 40% of cases,³ and diagnostic delays exceeding 5 years have also been demonstrated in this patient population.² For patients, such delays can be catastrophic: although there is no cure for ILD, which causes lung inflammation and permanent scars, treatments can slow progression and lessen symptom severity, allowing patients to live longer and with a higher quality of life.

In some instances, time lost can mean lung lost^2,4: In idiopathic pulmonary fibrosis (IPF), for example, which accounts for 17% to 37% of all ILD diagnoses,⁵ a delayed diagnosis can lead to irreversible loss of lung function and may eliminate certain treatment options, such as lung transplantation.²

To improve early diagnosis, a clinician toolkit and other resources were recently developed by a collaboration involving pulmonologists and primary care physicians (PCPs) from across the US.⁴ These experts comprise the steering committee for Bridging Specialties™: Timely Diagnosis for ILD, a joint initiative between the American College of Chest Physicians (CHEST) and the Three Lakes Foundation,¹ a nonprofit organization founded by a family tragically affected by lack of awareness and treatment options in ILD/IPF.

The clinician toolkit includes:

• an ILD-specific patient questionnaire;
• e-learning modules and a video gallery to increase provider knowledge of symptoms and radiologic features of ILD; and
• an interactive decision-making module to guide the examination, diagnosis, and treatment or referral of patients who may have ILD.

Additional resources available from the Bridging Specialties initiative include a white paper on factors influencing diagnostic delays in these patients and a podcast on overcoming barriers to ILD diagnosis.

To learn more about issues related to the timely diagnosis and treatment of ILD, we interviewed 2 members of the Bridging Specialties steering committee: William Lago, MD, family medicine physician at the Cleveland Clinic Wooster Family Health Center in Ohio; and Mary Beth Scholand, MD, FCCP, director of the Interstitial Lung Disease Clinic at the University of Utah in Salt Lake City.  

What are some of the key barriers to the timely diagnosis of ILD?

Dr Lago: Some of the barriers are the fact that ILD is relatively rare, and its symptoms are often vague and mild at presentation. They can often be confused with other, more common and immediate issues like heart disease or other serious issues. Patients may delay seeking treatment if the symptoms are mild enough as well. There have also been longstanding ideas among patients and even some providers that little can be done to help with the treatment of ILD, which can lead to further delays in seeking diagnosis and treatment. 

Dr Scholand: Recognizing an ILD requires an awareness of this possibility when a patient presents with a cough or shortness of breath with activity. Further, specialized studies such as a high-resolution CT scan are required to secure the diagnosis. Finally, access to pulmonologists and ILD specialty centers can be difficult due to geographic and time barriers. In summary, there are many steps required to recognize and then confirm an ILD diagnosis. 

What prompted the development of the ILD Clinician Toolkit by the Bridging Specialties steering committee?

Dr Lago: This was a project designed to increase awareness about ILD, aid PCPs in recognizing it earlier, and improve ILD diagnosis and treatment. Any time gained in getting patients diagnosed and beginning treatment can make significant differences in the lives of patients. 

Dr Scholand: The toolkit was devised to provide clinicians with accessible, efficient, and useful information to raise awareness of the entity of ILD and to improve confidence in the detection and management of an ILD. Unless the disease is on the forefront of a busy clinician’s radar, it will frequently be missed. In addition, many clinicians are unfamiliar with algorithms and resources to further manage a suspected ILD. The goal of the Bridging Specialties steering committee was to break down these barriers by bringing assistance to the fingertips of providers throughout a variety of specialties.

What are additional ways for clinicians to reduce the time to ILD diagnosis?

Dr Lago: The committee developed educational information to help clinicians improve their recognition of ILD, along with diagnostic tools they can use while seeing patients. We have also created information that physicians can share with patients to help them understand the importance of rapid diagnosis and treatment.

I think the most important thing is to put ILD on the radar of physicians, to include ILD in our differential diagnosis when looking at vague symptoms such as cough, mild shortness of breath, or fatigue. We can also utilize these tools to work with our pulmonary colleagues to help improve patient treatment and quality of life. 

Dr Scholand: Perhaps the most important element is recognition. The data support that these patients are left without a definitive answer to their concerns for at least 1-2 years.² If clinicians can recognize easy clinical clues such as persistent symptoms, crackles at lung bases, or oxygen desaturations, then they can move towards making a diagnosis more quickly.

Bringing together PCPs and pulmonologists, including ILD specialists, to rapidly identify and begin treatment for these patients has so many positive effects for patients, including alleviating the anxiety of an uncertain diagnosis and beginning therapies that can slow progression of the disease, allowing for long-term improvements in quality of life.

What other measures are needed to improve the overall landscape of care for ILD, and what is the potential role of the Bridging Specialties initiative in these endeavors?

Dr Lago: I think having more options and better treatments at our disposal could help patients who are devastated by diseases like pulmonary fibrosis. Any hope we can give these patients will have a huge impact on their health. 

With Bridging Specialties, CHEST and Three Lakes Foundation were able to bridge the gap between primary care and specialty care to help frontline providers improve their ability to diagnose and treat patients with rare but significant ILDs like pulmonary fibrosis. By working together, we can make a huge impact on our patients’ lives. 

Dr Scholand: There are so many more things we need to learn about ILD and pulmonary fibrosis. We have effective therapies that slow progression of fibrosis, but research is needed for better therapies that halt or regress fibrosis. We need to continue to improve our coordination amongst specialties to decrease the health care burden for patients with ILD.      

The Bridging Specialties initiative is innovative, as it gets to the very basis of patient care — understanding our patient’s needs and acting on them efficiently and effectively. ILD is an unusual etiology for common symptoms, but there are many patients suffering from this group of diseases. We are hopeful that our program, including the toolkit, will catalyze important changes in the lives of patients with ILD through early detection, better coordination of care, and improved therapies.