Systemic JIA with Features Concerning for MAS
Note: These treatment options are not meant to be mutually exclusive, and there may be
certain clinical situations for which the simultaneous initiation of more than one of these
medications is appropriate. Combination therapy with anakinra, a calcineurin inhibitor,
and systemic GCs was not specifically addressed.
Table 1. Initial Therapy for Systemic JIA with Features
Concerning for MAS
Treatment Quality of Evidence
Anakinra C
Calcineurin inhibitor C
Systemic GC
monotherapy
(IV or PO)
C
With the exception of continuing GC monotherapy for >2 weeks in
patients with continued features concerning for MAS, in which case it was
inappropriate (D).
Specific tapering strategies for GCs were not specifically addressed by the ACR.
Table 2. Uncertain or Inappropriate Options for Continued
MAS Activity
Treatment Quality of Evidence
Canakinumab Uncertain
With the exception of patients with an MD global <5 who had
received no prior therapy, GC monotherapy, or calcineurin
monotherapy, in which case it was inappropriate (D)
Rilonacept Uncertain
Tocilizumab Uncertain
Abatacept D
Inappropriate
IVIG D
Inappropriate
With the exception of patients who had tried a calcineurin inhibitor
in combination with anakinra, in which case it was uncertain.
MTX or leflunomide D
Inappropriate
Rituximab D
Inappropriate
TNF-α D
Inappropriate
With the exception of patients who had tried a calcineurin inhibitor
in combination with anakinra, in which case it was uncertain.
Treatment
