10
Treatment
Figure 3. Initital Treatment Options for the Treatment of
Interstitial Lung Disease Associated With Systemic
Autoimmune Diseases of Interest
* Decisions on GC dose and use of oral versus intravenous therapy depend on severity of disease. GCs
should be used cautiously in patients with MCTD with a systemic sclerosis phenotype who may be
at increased risk of renal crisis.
†
Treatments are listed in order based on a hierarchy established by head-to-head votes, although the
panel noted that decisions on which first-line therapy to use were dependent on specific situations
and patient factors. In all diseases, mycophenolate was conditionally recommended over the other
listed therapies. erapies here are divided into "preferred" options and "additional options" based
on the rank-order hierarchy.
Systemic Sclerosis
First-line ILD
therapy
Glucocorticoids
+
Preferred
Additional
options
Myositis
Strong recommendation
against glucocorticoids
(GCs)
Short-term GCs*
Mycophenolate
†
Tocilizumab
Rituximab
Mycophenolate
†
Azathioprine
Rituximab
CNI
Cyclophosphamide
Nintedanib
Azathioprine
Janus kinase inhibitor (JAKi)
Cyclophosphamide
