15
Figure 5. Recommendations for Management of SARD With
RP-ILD
Conditional recommendation
Rapidly-Progressive ILD
Intravenous (IV)
Glucocorticoids*
IV Glucocorticoids*
Consider early lung transplant referral
* In rare patients with systemic sclerosis with rapidly progressive ILD, there was no consensus on
whether or not to use glucocorticoids — if used, patients should be monitored closely for evidence
of renal crisis.
†
Rituximab and cyclophosphamide recommended over IVIG, but IVIG may be preferred if there is
high concern for infection.
+ +
NO
YES
Severe disease or
high suspicion for
anti-MDA5
1 or 2 additional therapies:
Rituximab
Cyclophosphamide
IVIG
†
Mycophenolate
Calcineurin inhibitor
JAKi
2 additional therapies:
Rituximab
Cyclophosphamide
IVIG
†
Mycophenolate
Calcineurin inhibitor
JAKi
