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.