New ACR Guidelines for Screening, Monitoring, and Treatment of SARD-ILD in Focus

The full manuscripts of the ACR guidelines for the screening, monitoring, and treatment of systemic autoimmune rheumatic disease (SARD) associated with interstitial lung disease (ILD) are expected to be published in the spring of 2024. The guideline summaries are available now.

Sindhu R. Johnson, MD, PhD, Professor at the University of Toronto, Canada, discussed the recommendations included in the guidelines during the ACR Convergence 2023 session, ACR Guidelines: Interstitial Lung Disease (ILD). The session is available on demand for registered ACR Convergence participants through October 31, 2024, on the meeting website.

The guideline conditionally recommends short-term glucocorticoids for all rheumatic diseases except for systemic sclerosis (SSc), where it is strongly recommended against, Dr. Johnson explained. If progression occurs, mycophenolate is conditionally recommended if not used as first-line therapy. In rapidly progressive ILD, combination therapy with intravenous glucocorticoids and two additional therapies is recommended.

“The guideline conditionally recommends mycophenolate as a first ILD treatment across the rheumatic diseases; however, there is a menu of treatment options that one may choose based on the individual patient,” Dr. Johnson said.

For patients at an increased risk of ILD, the guideline conditionally recommends two tests for screening, including pulmonary function testing (PFT) with spirometry, lung volumes, and diffusion capacity and high-resolution computed tomography (HRCT) of the chest. If the diagnosis is confirmed, three tests are conditionally recommended for monitoring, including PFT with all three components every three to six months for the first year, ambulatory desaturation testing every three to 12 months, and HRCT as needed.

Dinesh Khanna, MD, MSc, Professor at the University of Michigan, presented patient cases demonstrating the application of the guidelines.  

In one case, a 51-year-old female had a diagnosis of early diffuse scleroderma. She had a history of joint swelling and puffy fingers. Her modified Rodnan skin score was 23/51, suggesting significant skin fibrosis. Her chest was clear to auscultation. She had elevated platelet counts, elevated ESR/CRP anti-topoisomerase I antibodies, and a HAQ-DI score of 1.75, consistent with significant or moderate disability. Screening with PFTs confirmed a restriction in the forced vital capacity, and HRCT showed very early but definite ILD with ground opacity and a few interstitial markings.

The preferred therapies for this patient are mycophenolate, tocilizumab, and rituximab, Dr. Khanna explained. For progression, the use of nintedanib versus immunosuppression depends on the pace of progression and the amount of fibrotic disease or interstitial pneumonia pattern on HRCT.

Elana J. Bernstein, MD, MSc, Associate Professor at Columbia University Irving Medical Center, discussed the development of the SARD-ILD guidelines.

The process began with identifying 241 clinically relevant population, intervention, comparator, and outcomes (PICO) questions, followed by a systematic literature review. For each question, the certainty of evidence was evaluated using the GRADE methodology, and a 70% agreement was required to achieve consensus.

“A recommendation is considered strong if the voting panel is highly confident that the benefits of an intervention clearly outweigh the harms or vice versa. A recommendation is conditional if there’s uncertainty regarding the balance of benefits and harms,” Dr. Bernstein said.

The panel was permitted to make strong recommendations despite the low quality of evidence when the evidence suggested benefit in a life-threatening situation or potential catastrophic harm. This process resulted in 50 recommendations.