12
Treatment
Figure 4. Recommendations for Management of SARD-ILD
Progression Despite First-line ILD Treatment
* If intolerance leads to suboptimal dosing of first-line therapy consider switch to an alternative first-
line therapy.
†
erapies are generally listed in order based on a hierarchy established by head-to-head votes,
but decisions depend on specific clinical situations. Decision on whether to switch therapy or
add to current therapy depends on current therapy and on which therapy is being initiated.
Cyclophosphamide is not typically used in combination with other therapies, while others may be
used individually or in combination.
‡
Decision on use of nintedanib vs immunosuppression depends on pace of progression and amount
of fibrotic disease or presence of a usual interstitial pneumonia pattern on CT chest.
§
JAKi conditionally recommended as an option particularly in patients with anti-MDA-5.
¶
Short-term glucocorticoids may be of use in some patients with disease flares or as a bridge when
switching therapy.
Systemic Sclerosis
Therapy Options
Additional
Considerations
Myositis
Strong against
long-term GCs
Against
long-term GCs
¶
Mycophenolate
Rituximab
Nintedanib
‡
Tocilizumab
Cyclophosphamide
Autologous hematopoetic
stem cell transplant
(AHSCT) referral at
experienced center
Mycophenolate
Rituximab
CNI
Nintedanib
‡
Cyclophosphamide
IVIG
JAKi
§
