The Specialist Forum Volume 13 No 11 November 2013 | Page 41

ARTHRITIS

?? Organomegaly.
?? Low or absent natural killer cell activity.
?? Hyperferritinemia.
?? Central nervous system dysfunction.
According TOthe TFP, the definition was left intentionally broad because of the lack of validated classification criteria for MAS and exclude critically ill patients requiring admission to intensive care units.
• The TFP developed a separate ‘scenario’ for repeat tuberculosis
(TB) screening among all categories of JIA patients treated with
biologics. The TFP was asked to consider the appropriateness of
different screening approaches for TB such as:
?? Annual screening
?? Risk-based approaches
I
t is important to note that the TFP did not consider the method of
screening (e.g. interferon-?–release assay versus tuberculin skin test).

Drugs considered
The following drugs were included for consideration in the study:
• NSAIDs.
• Glucocorticoids (GC).
• Methotrexate.
• Leflunomide.
• Intravenous immunoglobulin (IVIG).
• Calcineurin inhibitors.
• Tumor necrosis factor-a (TNF-?) inhibitors.
• Abatacept.
• Rituximab.
• Anakinra.
• Canakinumab.
• Rilonacept.
• Tocilizumab.
The last three drugs were not included in the 2011 guideline. There
was no consideration of combination therapy with a biologic agent.
When biologic agents were evaluated as sequential therapy, it was
indicated that prior biologic agents were discontinued before initiation
of a new one.

Recommendations
Systemic JIA with active systemic features and
varying degrees of synovitis

Figure 1: Treatment pathways for patients with active systemic features and varying degrees of synovitis
Rheumatology Forum | November 2013

Initial therapeutic options
• Anakinra is recommended as a possible initial therapeutic option
for patients with a MD global ?5 irrespective of the AJC, or an MD
global <5 and an AJC >0.
• Systemic oral or intravenous (IV) GC monotherapy is recommended
for a maximum period of two weeks as a therapeutic option for
patients with an MD global <5 and an AJC >4 as well as for all
patients with an MD global ?5 irrespective of the AJC.
• Continuing GCs as monotherapy for more than one month is
recommended for patients with continued disease activity is
inappropriate.
• Initiating NSAID monotherapy in a patient without prior treatment is
recommended as one approach for patients with an MD global <5
irrespective of the AJC.
• NSAID monotherapy is recommended for patients with an MD
global ?5 and an AJC >0.
• Continuing NSAID monotherapy for longer than one month is not
recommended for patients with continued disease activity.

Therapeutic options for continued disease
activity
• Abatacept is recommended only for patients with an MD global ?5
and an AJC >4 after a trial of both an IL-1 inhibitor and tocilizumab
(sequentially).
• Abatacept is not recommended for patients with an AJC of 0
irrespective of the MD global, except in patients who had tried both
an IL-1 inhibitor and tocilizumab (sequentially).
• Abatacept is not recommended for patients with an MD global
<5 and an AJC >0 or an MD global ?5 and an AJC <4, except
in patients who had tried both an IL-1 inhibitor and tocilizumab
(sequentially) or a DMARD plus either an IL-1 inhibitor or tocilizuma.
• Abatacept is not recommended for patients with an MD global
?5 and an AJC >4, except in patients who had tried both an IL-1
inhibitor and tocilizumab (sequentially) or patients who had tried a
DMARD plus either an IL-1 inhibitor or tocilizumab.
• Anakinra is recommended for patients with continued disease
activity after treatment with GC monotherapy or NSAID monotherapy.
• The use of a calcineurin inhibitor is recommended only for patients
with an MD global ?5 and an AJC of 0 after a trial of both an IL-1
inhibitor and tocilizumab (sequentially).
• The use of a calcineurin inhibitor for patients with an MD global
<5 and an AJC of 0 is not recommended, with the exception of
patients who received either an IL-1 inhibitor or tocilizumab.
• The use of a calcineurin inhibitor is not recommended for patients
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