New ACR SSc-ILD guidelines warn against glucocorticoids as first-line therapy
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Key takeaways:
- Newly announced ACR guidelines recommend against glucocorticoids as first-line therapy for interstitial lung disease in patients with SSc-ILD.
- The full recommendations — with options for screening, monitoring and treating ILD — will be published early next year.
The American College of Rheumatology “strongly recommends” against the use of glucocorticoids as a first-line therapy for interstitial lung disease in patients with systemic sclerosis-associated ILD, according to newly released guidelines.
The new guidelines also include recommendations for the screening, monitoring and treatment of patients with, or who are at-risk for, ILD. The ACR anticipates the guidelines will appear in a peer-reviewed rheumatology journal in early 2024, a press release announcing the recommendations said.
“Interstitial lung disease is a major cause of morbidity and mortality across several systemic autoimmune rheumatic diseases,” Sindhu R. Johnson, MD, PhD, director of the clinical epidemiology and health care research program at the University of Toronto, and lead author of the new guidelines, said in the release. “Guidance was needed for which tests to use for screening and monitoring this particular disease.”
The new guidelines are intended to offer guidance to physicians caring for patients with complex diseases, comprising multiple potential therapies and monitoring options, the release noted.
The ACR guidelines include eight recommendations regarding disease screening, 11 for disease monitoring, 17 for first-line management of ILD, 12 for disease management in patients for whom the first-line therapy failed, and three recommendations for patients who have “rapidly progressive ILD.” An advance selection of recommendations highlighted by the ACR follow:
- It is conditionally recommended that providers screen patients at “high risk” for developing ILD with high-resolution computed tomography (HRCT) of the chest or pulmonary function tests (PFTs), or both.
- It is conditionally recommended that providers monitor disease progression using HRCTs and/or PFTs.
- It is conditionally recommended that providers monitor disease progression with ambulatory desaturation testing.
- It is conditionally recommended that physicians refrain from screening or monitoring disease with a 6-minute walk test distance, chest radiography or bronchoscopy.
- Patients with systemic autoimmune rheumatic disease-related ILD — aside from SSc-ILD — can receive a glucocorticoid in short durations as a first-line therapy.
- For patients with SSc-ILD, it is strongly recommended that patients do not receive glucocorticoids as a first-line therapy for ILD.
- Potential first-line therapy options for patients with SSc-ILD include mycophenolate, rituximab (Rituxan, Genentech), cyclophosphamide and azathioprine.
- It is recommended that patients do not receive leflunomide, methotrexate, tumor necrosis factor inhibitors or abatacept (Orencia, Bristol Myers Squibb) as first-line therapy options.
- If SARD-ILD progresses following first-line therapy, patients may receive mycophenolate, rituximab, cyclophosphamide or nintedanib (Ofev, Boehringer Ingelheim).
- Among patients with Sjogren’s-related ILD and idiopathic inflammatory myopathy-related ILD who progress despite initial ILD therapy, it is conditionally recommended these patients do not receive tocilizumab (Actemra, Genentech).
“In those with systemic sclerosis-ILD, we strongly recommend against using glucocorticoids as a first-line ILD therapy or after ILD progression, because glucocorticoids confer an increased risk of scleroderma renal crisis,” Johnson said in the release. “Given the moderate certainty of the evidence for harm and low certainty of the evidence for benefit, we voted strongly against using this treatment for patients with systemic sclerosis-ILD.”
According to the release, the ACR developed these guidelines with “the best available evidence and consensus across a range of expert opinions and incorporated patient values and preferences,” with the intention of minimizing regional variation in patient care. The full guideline summaries can be found on the ACR’s website.
“We know that early detection and hastened referral to care, in collaboration with pulmonology, is critical for the best patient outcomes,” Sonye K. Danoff, MD, PhD, a pulmonologist and director of the ILD and pulmonary fibrosis program at Johns Hopkins University School of Medicine, said in the release.
“Because symptoms of ILD (cough, shortness of breath, fatigue) can be subtle or result from other common diseases, the diagnosis of ILD can be delayed,” she added. “Increasing awareness of the groups at highest risk for developing ILD and implementing appropriate screening and treatment practices should have long-term benefits.”
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ACR press release
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