9
Table 6. Recommendations for Management of SARD-ILD
Progression Despite First-line ILD Treatment
Recommendations
For people with SSc-ILD progression despite first ILD treatment, we strongly
recommend against using long-term glucocorticoids, and in other SARD-ILD we
conditionally recommend against using long-term glucocorticoids.
For people with SARD-ILD progression despite first ILD treatment, we conditionally
recommend mycophenolate, rituximab, cyclophosphamide, and nintedanib as treatment
options.
For people with RA-ILD progression despite first ILD treatment, we conditionally
recommend adding pirfenidone as a treatment option.
For people with SARD-ILD, other than RA-ILD, progression despite first ILD
treatment, we conditionally recommend against adding pirfenidone as a treatment
option.
For people with SSc-ILD, MCTD-ILD, RA-ILD progression despite first ILD
treatment, we conditionally recommend using tocilizumab as a treatment option.
For people with SjD-ILD and IIM-ILD progression despite first ILD treatment, we
conditionally recommend against using tocilizumab as a treatment option.
For people with IIM-ILD progression despite first ILD treatments, we conditionally
recommend using a calcineurin inhibitor as a treatment option.
For people with SARD-ILD, other than IIM-ILD, progression despite first ILD
treatments, we conditionally recommend against using a calcineurin inhibitor as a
treatment option.
For people with IIM-ILD progression despite first ILD treatment, we conditionally
recommend using JAK inhibitors as a treatment option.
For people with IIM-ILD and MCTD-ILD progression despite first ILD treatment, we
conditionally recommend adding IVIG as a treatment option.
For people with SARD-ILD progression despite first ILD treatment, we conditionally
recommend against using plasma exchange as a treatment option.
For people with SSc-ILD progression despite first ILD treatment, we conditionally
recommend referral for stem cell transplantation and/or lung transplantation.
