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Interstitial Lung Disease in People With Systemic Autoimmune Rheumatic Disease

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8 Treatment Table 5. Summary of Recommendations for Management of SARD-ILD: First-line ILD Treatment Recommendations For people with SARD-ILD other than SSc-ILD, we conditionally recommend glucocorticoids as a first-line ILD treatment. For people with SSc-ILD, we strongly recommend against glucocorticoids as a first-line ILD treatment. For people with SARD-ILD, we conditionally recommend mycophenolate, azathioprine, rituximab, and cyclophosphamide as first-line ILD treatment options. For people with SSc-ILD and MCTD-ILD, we conditionally recommend tocilizumab as a first-line ILD treatment option. For people with SARD-ILD, we conditionally recommend against leflunomide, methotrexate, tumor necrosis factor inhibitor (TNFi), and abatacept as first-line ILD treatment options. For people with SSc-ILD, we conditionally recommend nintedanib as a first-line ILD treatment option. For people with SjD-ILD, IIM-ILD, and MCTD-ILD, we conditionally recommend against nintedanib as a first-line ILD treatment option. For people with RA-ILD, the Panel was not able to come to consensus on whether to recommend nintedanib as a first-line ILD treatment option. For people with SARD-ILD, we conditionally recommend against pirfenidone as a first- line ILD treatment option. For people with SARD-ILD receiving mycophenolate without evidence of ILD progression, we conditionally recommend against adding nintedanib or pirfenidone to mycophenolate. For people with SARD-ILD, we conditionally recommend against upfront combination of nintedanib or pirfenidone with mycophenolate over mycophenolate alone as first-line ILD treatment options. For people with IIM-ILD, we conditionally recommend JAK inhibitors as a first-line ILD treatment option. For people with SARD-ILD other than IIM-ILD, we conditionally recommend against JAK inhibitors as a first-line ILD treatment option. For people with IIM-ILD, we conditionally recommend calcineurin inhibitor (CNI) as a first-line ILD treatment option. For people with SARD-ILD other than IIM-ILD, we conditionally recommend against CNI as a first-line ILD treatment option. For people with SARD-ILD, we conditionally recommend against intravenous immune globulin (IVIG) or plasma exchange as first-line ILD treatment options. For people with SARD-associated ILD, we conditionally recommend optimal medical management over referral for stem cell or lung transplant as first-line ILD treatment options.

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