Gaps Persist in Interstitial Lung Disease Care

Interstitial lung diseases (ILD) are a diverse group of pulmonary disorders, some of which are difficult to diagnose. Patients often face significant hurdles from the onset of symptoms to diagnosis to treatment costs, many of which physicians aim to overcome through shared decision-making.

The Washington, DC, Pop Health Roundtable, hosted by The American Journal of Managed Care^®, aimed to bridge gaps across health care systems and open communication across specialties to improve patient outcomes and access to value-based care. This particular roundtable discussion focused on ILD and persistent gaps clinicians face.

The discussion featured 13 experts who specialized in various areas of disease management like rheumatology, pulmonology, and pharmacy. Panelists discussed hurdles they often face when managing patients with ILD and how the different strategies, applied at their respective practices, might inspire others to do the same in hopes that it would bring them closer to the goal at hand: better quality of life for patients.

Delays in Diagnosis and Access Persist Across ILD Care

Steven D. Nathan, MD, moderator and Schar Chair, Advanced Lung Disease and Lung Transplant programs, Inova Fairfax Hospital, started the conversation, asking the panelists how they’d characterize overall disease burden for patient populations and individuals.

Christopher S. King, MD, associate medical director, Advanced Lung Disease and Transplant Program, and director, Pulmonary Fibrosis Foundation, at Inova Fairfax Hospital, responded, exemplifying the difference in referrals he sees for various ILDs at his institution. Although there may be more ILD patients in the community, and at Inova Fairfax Hospital, King said they often see fibrotic ILDs more than inflammatory ILDs, and more specifically, among older patients.

“I think we see a very skewed population of patients who get referred to us. Are we seeing the tip of the iceberg?” Nathan echoed. “I don't know that we ever will know the answer to that, but I think if you look across the US at least, there's an increasing incidence of pulmonary fibrosis in general, and it might be to your point that we're getting more CTs, so we're recognizing it more.”

Nathan then pivoted to hear a rheumatologist’s perspective, as they’re often screening for ILDs in patients, as the symptoms of ILDs and rheumatology diseases often overlap.

Lauren Smith, MD, a rheumatologist at MedStar Georgetown, said that she screens all her patients with CT or pulmonary function tests (PFTs), pointing out a few conditions that make her extra cautious of a potential ILD diagnosis, like rheumatoid arthritis, a new cough, or decreased exercise tolerance.

Megan Lockwood, MD, a rheumatologist specializing primarily in lupus at Georgetown, responded to Smith’s statement, adding more rheumatology diseases that are important to screen for in patients potentially at risk for ILDs.

“I probably think about it more in some of our Sjögren's patients and make sure that we're being more mindful of screening for it,” she said. “But somewhat controversially, in [the] Sjögren's management guidelines that came out a few years ago, it was actually recommended that every patient with Sjögren’s get an annual screening chest X-ray and/or a CT scan.”

Although Lockwood pointed out that the screening guideline, specifically for patients with Sjögren’s, may be “overkill,” Smith said PFTs may be more reasonable as an annual test when considering radiation exposure.

In contrast, Jamal A. Mikdashi, MD, MPH, a rheumatologist at the University of Maryland, supported the use of CT as a regular screening method, despite absent guideline recommendations.

“Now, because of the high resolution of the CT, the diagnosis is becoming easier, and detection is now easier, and therefore the prevalence of ILD is going up,” he said. “But in our condition, we are not putting any guidelines into screening. That's a limitation. We start to look into it, but there are no recommendations from our societies at the national level.”

Nathan highlighted that despite advances in antifibrotic therapies, patients continue to experience lengthy delays between symptom onset and diagnosis. He cited significant increases in median survival in idiopathic pulmonary fibrosis (IPF), progressive pulmonary fibrosis (PPF), and rheumatoid arthritis–associated ILD with the use of antifibrotic therapy.¹ However, despite the demonstrated efficacy of these therapies in ILD, the delay from symptom onset to ILD diagnosis is almost 12 months, he said.

Some panelists attributed the gap to patients waiting to report symptoms; others, like Anju Singhal, MD, an Advanced Lung Program physician and director of the Cystic Fibrosis and Bronchiectasis Program at Inova Fairfax Hospital, underscored the complexities patients face when navigating the health care system.

“Getting an appointment with your primary care [physician], getting the referral, finding the referral doctor, [and then] getting in, I think, contributes a lot to delay as well,” she said.

Patient History and Early Recognition Remain Critical

The panel also discussed how incomplete exposure histories and limited appointment times can delay identification of patients with IPF or PPF. Nathan asked the panelists how far into a patient’s history they are meeting with them and what type of questions they’re asking to truly gauge whether a patient may be at risk for an ILD such as IPF or PPF.

Some panelists, like Shambhu Aryal, MD, medical director of the Lung Transplant and Sarcoidosis Program at Inova Fairfax Hospital, said that as a tertiary partner, a lot of his patients are more advanced in their disease than some other institutions. He also highlighted that many patients with ILD are “lingering around with primary care and not really getting the care they need.”

Speaking to Aryal’s point, Mikdashi noted that primary care patient history and exposure are not as detailed or extensive compared with that of pulmonary physicians, suggesting another reason for referral, diagnosis, or treatment delays. Nathan affirmed Mikdashi’s comment, acknowledging the routine exposure questions that aid in diagnosing patients at risk for ILD. Another panelist pointed out the time constraints that may make these questionnaires difficult to complete for primary care physicians.

Alan Nyquist, MD, an advanced lung disease and lung transplant physician, fellowship site director, and associate program director, Cystic Fibrosis and Bronchiectasis Program at the Inova Advanced Lung Disease and Transplant Program, at Inova Fairfax Hospital, emphasized that patient–clinician interactions vary between institutions.

“Some places have 35, 40, or 50 minutes with patients,” he said. “Other practices have 10 minutes, so you can't ask all those ILD-specific questions, and that's another way that you will miss diagnoses.”

The conversation continued to pinpoint microcosms within clinical practice that often delay diagnosis or treatment that eventually exacerbate patients’ disease risk and development. Nathan asked Jared D. Wilkinson, MD, an advanced lung disease and lung transplant physician and Alpha-1 medical director at Inova Fairfax Hospital, how primary care physicians may misinterpret PFTs and what the downstream effects are on patient outcomes and disease trajectory.

“For the patient, that impacts tremendously and can mean the difference between returning to a more functional baseline vs having significant long-term impairments,” Wilkinson said.

Nathan used Wilkinson’s example of diagnosis and treatment delays as a segue into associated cost burdens and asked panelists how they navigate that barrier while balancing patients’ needs and value-based care.

Singhal, in response, pointed out the treatment approach in ILD, which often uses a prevention-of-progression care model compared with cystic fibrosis or bronchiectasis, which uses a general prevention strategy.

Treatment Decisions Depend on Tolerability and Disease Progression

As the discussion progressed, the panelists found themselves on the topic of antifibrotic therapies and differences in treatment strategies between rheumatologists and ILD physicians.

Zachary Harris, PharmD, pharmacy supervisor of retail and specialty pharmacy at Inova Fairfax Hospital, said that the majority of patients referred from Inova’s rheumatology clinic are not referred for antifibrotic therapies.

“What we may see is patients coming through general pulmonologists who have something like treatment-resistant asthma,” Harris said. “They may end up on biological therapies. They may end up on [an] inhaler. But in terms of antifibrotics coming out of rheumatology, it's just not a volume we see.”

Yet, there are varying factors that physicians use to guide their treatment strategy, particularly regarding antifibrotics such as nerandomilast, nintedanib, pirfenidone, and tocilizumab.

“In terms of which factors guide treatment selection, I would say, for most of the prescribing that I see, it's what the patient will tolerate,” Nyquist said. “If we look at what's the most antifibrotic therapy, it's what the patient can stay on, what they can tolerate, and what they'll continue long-term.”

Insurance Barriers Continue to Shape ILD Treatment

As Nyquist described his prescription patterns, he noted that insurance barriers make it difficult to properly manage a patient’s disease. Many of the panelists shared these sentiments, explaining that they get frequent payer requests for step therapy after being denied for adjunctive therapy.

Nyquist said he’s seen insurers deny claims for nerandomilast adjunct therapy for a patient stable on nintedanib because they weren’t given pirfenidone first.

“But nerandomilast is slowly becoming almost bread and butter, and we're switching to nintedanib if insurance is denying,” Nyquist said. “And pirfenidone…was probably the least prescribed antifibrotic therapy I've seen.”

The Pop Health Roundtable discussion concluded with the panelists summarizing their talking points related to multidisciplinary care, treatment strategy, access barriers, and delays in diagnosis and treatment. Their final sentiments landed on the need for open and strengthened communication across specialties and multidisciplinary clinics to advance patient outcomes and disease management.

References

1. Orser AG, Pooler C, Archibald N, et al. The diagnostic pathway for patients with interstitial lung disease: a mixed-methods study of patients and physicians. BMJ Open Respir Res. 2024;11(1):e002333. doi:10.1136/bmjresp-2024-002333