Patients with progressing SSc-ILD should be seen ‘every 3 to 6 months’
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SAN DIEGO — Rheumatologists should stratify patients with systemic sclerosis-associated interstitial lung disease into phenotypes in order to closely monitor those with progressing disease, according to a speaker here.
“One thing that can be helpful in your practice is to have some kind of method for characterizing phenotypes of your patients,” Elizabeth R. Volkmann, MD, MS, director of the scleroderma program at the University of California Los Angeles, told attendees at the 2023 Congress of Clinical Rheumatology West meeting. “This is a very simple sort of way that I look at it.”
According to Volkmann, patients with SSc-ILD can, in the simplest terms, typically be stratified into four groups — those demonstrating an improvement in their disease, those with stable disease, patients who are slowly progressing, and those who are quickly progressing.
The most important groups to closely monitor are the patients with slowly or rapidly progressing disease, she added.
“These are patients where you don’t just want to see them every year — you want to see them every 3 to 6 months,” Volkmann said.
The first step in this process involves establishing a baseline for symptoms and disease activity. After a CT scan confirms the diagnosis, it is time to check in with patients to evaluate their symptoms, according to Volkmann.
“You’ve gotten your CT scan of the chest, you know that they have ILD, but you want to check on their symptoms,” she said.
Volkmann recommended asking patients about dyspnea and coughing symptoms, as well as evaluating their pulmonary function results. Additionally, rheumatologists should establish the radiological extent of disease and consider how the patient may be impacted by symptoms unrelated to SSc-ILD, she said.
“Pulmonary function tests, while they are not a sensitive way to detect ILD, are a good way to stage the disease,” Volkmann said. “You want to establish their baseline forced vital capacity and their baseline [diffusing capacity of the lungs for carbon monoxide].
“Also, critically, consider the whole patient in this assessment,” she added. “Are there other clinical dimensions of this patient’s disease that might influence their walk test or their cough?”
The next step for being able to characterize phenotypes requires an understanding of the definitions of progressive pulmonary fibrosis. According to Volkmann, this is typically defined as a patient meeting two of three criteria — worsening respiratory symptoms due to ILD, physiologic decline or any radiological progression.
Once a patient is diagnosed with ILD, it is likely that the rheumatologist will be working with a pulmonologist in the management of the disease, she added. At this point, it is important to personalize a monitoring strategy for each patient.
“I try to really stay engaged, because I think this is the time we can really intervene as rheumatologists and really alter this patient’s treatment course,” Volkmann said. “I recommend in patients with early disease, to get their pulmonary function tests every 3 to 6 months, because you need different data points to establish a trend.”
It is also important to decide how often patients will repeat their CT scans, she added.
“You also want to decide how often you will repeat the CT scan,” Volkmann said. “Many people have this concern that you don’t want to repeat the CT scan too often because of radiation exposure, and this is very reasonable, but with the CTs we use now for monitoring ILD, there are low-dose protocols you can use.”
Early in the course of disease, Volkmann stated she typically aims for annual scans, she said.
“When you characterize these patients, the only way to develop these phenotypes and understand them is to follow these patients closely,” she said.
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Volkmann ER. Systemic sclerosis associated ILD: How to evaluate subclinical, clinical and progressive disease for best outcomes. Presented at The Congress of Clinical Rheumatology West; Sept. 7-10, 2023 (hybrid meeting).
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