Rheumatology, pulmonology must support each other in managing interstitial lung disease

AUSTIN, Texas — Ongoing communication between rheumatology and pulmonology is essential to managing “unpredictable” interstitial lung disease, according to a presenter at the 2023 Rheumatology Nurses Society annual conference.

“ILD is a huge topic,” Cori Fratelli, BSN, MSN, FNP-BC, a nurse practitioner in the division of pulmonary, critical care and sleep medicine at National Jewish Health, in Denver, Colorado, told attendees. “Collaboration is important.”

However, Fratelli suggested that communication between rheumatology and pulmonology regarding these patients is often less than perfect.

“Teamwork [should be] a standard, not an ideal,” she said.

Although there is an umbrella of approximately 200 diseases that impact the interstitium, there are three symptoms that can signify autoimmune ILD, according to Fratelli. They are dry cough, dyspnea and crackles in the lungs that “sound like Velcro,” Fratelli said.

“If you hear crackles, I would like you to a walk down the hall,” she added.

Fratelli noted that rheumatology is often the “first touchpoint” before patients with systemic sclerosis- and RA-associated ILD are sent to pulmonology. However, a particular concern is that when rheumatology providers suspect ILD in their patients, they often refer them to pulmonology immediately, before taking any kind of action.

“Rheumatology providers do not often get to interact with ILD testing in a way that makes sense,” Fratelli said.

For a patient with suspected ILD, rheumatologists may check for gas exchange using a 6-minute walk test, or check the patient’s forced vital capacity (FVC) or forced expiratory volume (FEV1), which calculates the amount of air that a person can force out of their lungs in 1 second. Rheumatologists should consider learning the parameters of normal and abnormal values for FEV1, FVC and the ratio between the two measures, Fratelli said.

“When a patient comes to you and says, ‘I get short of breath when I get up to walk,’ you should be worried,” she said.

A rheumatology provider may also order spirometry, diffusing capacity of the lungs (DLCO) and/or a chest X-ray.

“DLCO tells me how well a patient transfers oxygen from their lungs to their blood vessels,” Fratelli said. “A DLCO value under 80% could mean ILD.”

Although a chest X-ray is not an appropriate way to diagnose or evaluate ILD, it can be a broad way of answering one key question: “Does my patient have a lung issue?”

Another concern for Fratelli is that patients with ILD can be misdiagnosed with pneumonia.

“They get antibiotics but it gets worse,” she said. “Pneumonia looks fluffy on X-ray, like someone put cotton balls into your lungs. ILD looks like someone took little pieces of paper and threw them into your lungs.”

Once a patient with SSc- or RA-associated ILD has been diagnosed, collaboration is essential, according to Fratelli.

“Our rheumatology colleagues, we ask them to focus on the rheumatologic aspects of their disease,” she said.

Rheumatology providers have a better understanding of immunomodulation, autoantibodies and genetic testing than their pulmonary colleagues, Fratelli said. In addition to dealing with the lungs and antifibrotic medications, pulmonologists often coordinate mental health services and end-of-life care, if needed.

“This is the ideal collaboration,” Fratelli said.

Mastering this collaboration can be critical for one important reason, she added.

“Individual patient course can be unpredictable,” she said. “Rheumatology and pulmonology should develop an individual treatment plan based on evolving risk. Pivot as needed.”

Additionally, “constant conversations” should form the basis of all treatment decisions, according to Fratelli.

“Let me know how best to communicate with you,” she said, noting that text, phone calls, emails or messages via electronic health records are all acceptable forms of communication.

“Teamwork makes the dream work,” Fratelli added. “Medicine is no longer a silo — it is like a whole working farm.”