14
Treatment
Table 7. Recommendations for Management of SARD With
Rapidly Progressive ILD (RP-ILD)
Recommendations
For people with SARD and RP-ILD, we conditionally recommend pulse intravenous
methylprednisolone as a first-line RP-ILD treatment.
For people with SARD and RP-ILD, we conditionally recommend rituximab,
cyclophosphamide, IVIG, mycophenolate, CNI, and JAK inhibitors as first-line RP-ILD
treatment options.
For people with SARD and RP-ILD, we conditionally recommend against
methotrexate, leflunomide, azathioprine, TNF inhibitors, abatacept, tocilizumab,
nintedanib, pirfenidone, and plasma exchange as first-line RP-ILD treatment options.
For people with RP-ILD, we conditionally recommend up-front combination therapy
(triple therapy for those with confirmed or suspected MDA-5 and double or triple
therapy for those without confirmed or suspected MDA-5) over monotherapy as first-
line treatment.
For people with SARD and RP-ILD, we conditionally recommend against referral
for stem cell transplantation over optimal medical management as a first-line RP-ILD
treatment.
For people with SARD and RP-ILD, we conditionally recommend early referral for lung
transplantation over later referral aer progression on optimal medical management.
