ACR advises against glucocorticoids for interstitial lung disease in systemic sclerosis
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Key takeaways:
- ACR and the American College of Chest Physicians jointly recommend against glucocorticoids as first-line or long-term treatment of SSc-ILD.
- Glucocorticoids were conditionally recommended for all other SARDs, but only short term.
New recommendations from the American College of Rheumatology and the American College of Chest Physicians strongly advise against glucocorticoids for interstitial lung disease in systemic sclerosis.
However, the document conditionally recommends glucocorticoids as first-line ILD treatment in all other systemic autoimmune rheumatic diseases. The recommendations, published in Arthritis & Rheumatology, are the first for the treatment of ILD among people with SARDs to be endorsed by the ACR and American College of Chest Physicians.
“Interstitial lung disease is a significant cause of morbidity and mortality in people with systemic autoimmune rheumatic diseases (SARDs),” Sindhu R. Johnson, MD, PhD, a rheumatologist at the University Health Network and Sinai Health Systems, in Toronto, and colleagues wrote.
“This guideline provides recommendations for the treatment of ILD in adults with those SARDs at greatest ILD risk, including systemic sclerosis, rheumatoid arthritis, idiopathic inflammatory myopathies (including polymyositis, dermatomyositis, antisynthetase syndrome, immune-mediated necrotizing myopathy), mixed connective tissue disease and Sjögren’s disease.”
To draft new recommendations for the treatment of ILD in SARDs, a core leadership team developed research questions, while another team conducted a systematic literature review. The certainty of all the evidence was categorized as “low to very low,” the authors wrote.
In virtual meetings, an expert voting panel — consisting of 19 rheumatologists, four pulmonologists, a radiologist and three representatives from a patient panel — discussed the evidence from the literature review. The group “voted on paired comparisons of treatment options, resulting in a hierarchy of preferred treatments,” expressed as a range of “preferred” or “additional” options, the authors wrote.
According to the recommendations, providers should avoid using glucocorticoids to treat SSc-ILD due to the risk for scleroderma renal crisis. Strong recommendations were made against glucocorticoids both as first-line treatment and as long-term treatment after progression in SSc-ILD.
Meanwhile, glucocorticoids were conditionally recommended as first-line treatment in all other SARDs, but not for long-term use. Instead, they should be “reserved for short-term use, such as bridging to another therapy,” the authors wrote
Treatment options conditionally recommended for ILD in all SARDs included mycophenolate, azathioprine (Imuran, GlaxoSmithKline), rituximab (Rituxan, Genentech) and cyclophosphamide. Methotrexate, leflunomide (Arava, Sanofi), TNF inhibitors and abatacept (Orencia, Bristol Myers Squibb) were conditionally recommended against as first-line treatment, though they “may be appropriate for extrapulmonary manifestations,” according to the recommendations.
“Patient- and disease-specific factors may lead to selection of a different treatment within the ‘menu of options’ provided,” Johnson and colleagues wrote. “We provide this hierarchy to be transparent about how experts consider these options, particularly in the setting of low- to very low-certainty evidence.
“Because most recommendations are conditional, shared decision-making that accounts for factors such as ILD severity, risk factors for progression, other disease manifestations, cost and toxicity is crucial when choosing a medication within the range of recommended options,” they added. “Co-management with pulmonologists is advised for initiation of ILD treatment, particularly to determine the need for treatment in asymptomatic patients with stable and mild ILD.”
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