Interstitial lung disease (ILD) can be an underappreciated complication of most rheumatic diseases. The prevalence can vary from 5% to 60% or more, depending on the patient, the disease, and environmental and other factors.
“ILD is something we should assume is going to happen,” said Justin Oldham, MD, PhD, Associate Professor of Internal Medicine and Director of ILD Research at University of Michigan Medicine. “Depending on the autoimmune condition we’re talking about, ILD can range from rare to quite common. But when present, it can become a big problem, and it is always something that clinicians need to keep at the forefront of their connective tissue disease (CTD) management.”
Dr. Oldham will discuss the latest advances in optimizing monitoring and multidisciplinary care for progressive pulmonary fibrosis in patients with rheumatic disease during The Many Faces of ILD in Rheumatic Diseases, 1–2 p.m. on Monday, October 27, in Room W375D-E of McCormick Place. Erin Wilfong, MD, PhD, Assistant Professor of Rheumatology at Vanderbilt University Medical Center, will explore the latest approaches combining clinical and biological features of ILD in patients’ systemic autoimmune rheumatic diseases (SARDs) to improve management decisions.
“We know that ILD is common in many of our rheumatic diseases, and we know that it’s one of the biggest drivers of both morbidity and mortality for patients,” Dr. Wilfong said. “The question becomes, how do we best treat these patients? How do we think about who we need to be screening for lung disease, and how do we think about patients who don’t respond to first-line therapy? These are the three biggest issues right now.”
Rheumatic disease phenotype matters, she added.
Immunosuppression is a reasonable starting point for ILD associated with rheumatoid arthritis, with antifibrotics as a useful adjuvant for some patients.
Patients with systemic sclerosis-associated ILD may do better with combination treatment. There are caveats to the widely used mycophenolate/tocilizumab combination, and potential safety benefits and therapeutic synergy from mycophenolate/nintedanib. Growing evidence supports the early use of Janus kinase (JAK) inhibitors in ILD associated with anti-melanoma differentiation-associated gene 5 (MDA-5), and lung transplantation is an option for select patients with ILD associated with idiopathic inflammatory myopathies.
Dr. Oldham noted that, over the past year, the ACR has published two sets of guidelines with explicit calls for systematic screening for ILD and other lung disease in specific patient populations with autoimmune disease. There are also a number of exciting prognostic biomarkers to predict which patients are likely to develop progressive ILD and therefore most likely to benefit from treatment.
There is also the prospect of the first new drug for ILD since antifibrotics were approved more than a decade ago.
The recent FIBRONEER-IND trial of nerandomilast showed significantly slower decline in lung function for patients with progressive pulmonary fibrosis compared to placebo. Nerandomilast is an oral preferential inhibitor of phosphodiesterase 4B with both antifibrotic and immunomodulatory properties. The agent has already been shown to slow the progression of idiopathic pulmonary fibrosis.
“Nerandomilast will undoubtedly make its way into this session,” Dr. Oldham said. “Clinical trials show it effectively slows progression of ILD irrespective of the subtype, which included a large number of autoimmune conditions.”
Dr. Wilfong said there will be a significant focus on how to think about combination therapy.
“We need to think about the immune system to target it in multiple ways and the risks of doing that,” she said. “And we need to be thinking about prophylaxis to prevent infections in these patients.”
On-demand access to recorded presentations will be available to registered participants of ACR Convergence following the annual meeting through October 31, 2026.
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If you weren’t able to make it to a live session during ACR Convergence 2025 — or you want to revisit a session from the annual meeting — make plans to watch the replay. All registered participants receive on-demand access to scientific sessions after the meeting through October 31, 2026.