The Specialist Forum Volume 13 No 11 November 2013 | Page 40

ARTHRITIS

New systemic JIA guideline

R

esearch has shown that systemic juvenile idiopathic arthritis
(JIA) – arthritis in one or more joints for at least six weeks in
children 16-years or younger and accompanied or preceded
by fever for at least two-weeks that is daily for at least three of those
days, with one or more of the following symptoms: red rash, enlarged
liver, spleen or lymph nodes, and inflammation of the tissue lining of
the lungs, heart, or stomach - accounts for 4% to 15% of all JIA cases.
According to Manners and Bower the global reported prevalence of
JIA is estimated to be between 0.07 to 4.01 per 1000 children, while
the annual incidence is reported as 0.008 to 0.226 per 1000 children.
Possible reasons for the differences in these estimates include
diagnostic difficulties, development of new diagnostic criteria and
differing definitions of clinical cases, differences in case ascertainment (community based versus clinical case studies, qualification and
experience of study clinicians, definition of study population), changes
in standard of living, health care resources, increasing knowledge as
well of small studies and hence more chance fluctuation (Manners
and Bower).

The major variation in reported prevalence was due to the difference between true community based studies involving children from
within classrooms or homes (and not necessarily previously diagnosed with JIA) compared with clinical case studies of children who
(by definition) had been previously diagnosed. The highest prevalence
was reported for true community based studies.
The American College of Rheumatology (ACR) recently published
its new treatment recommendations for children with systemic JIA.
The recommendations were developed using the RAND/University
of California, Los Angeles Appropriateness Method. The objectives
of the new guideline are to update the 2011 ACR recommendations
for the use of nonbiologic disease-modifying antirheumatic drugs
(DMARDs) and biologic DMARDs in the treatment of systemic JIA.
The specific aims of the project were to:
• Develop treatment recommendations for patients with the following three general systemic JIA phenotypes: significant systemic
features and varying degrees of synovitis, sig nificant arthritis and
no significant systemic features, and features concerning for macrophage activation syndrome (MAS).
• Update the 2011 ACR recommendations regarding indications for
starting nonbiologic DMARDs and biologic DMARDs for systemic
JIA and indications for switching between non­ iologic DMARDs
b
and biologic DMARDs for systemic JIA.
• Incorporate the use of anti–interleukin- (IL) 1 and anti–IL-6 therapies
into the ACR recommendations for the treatment of systemic JIA.

JIA phenotypes
• First phenotype (systemic JIA with active systemic features and
varying degrees of synovitis): Active systemic features were defined
as the presence of any combination of the following:
?? Fever
?? Evanescent rash
?? Lymphadenopathy
?? Hepatomegaly
?? Splenomegaly
?? Serositis
The Task Force Panel (TFP) was asked to consider the treatments
among patients with a disease activity score (MD global) of <5 or ?5
on a 10-point numerical rating scale and by ankle joint complex (AJC)
(zero joints, one to four joints, or more than four joints).
• Second phenotype (systemic JIA without active systemic features
but with active synovitis): the appropriateness of medical therapies
based on the total number of active joints (four joints or more) was
rated.
• Third phenotype (systemic JIA with features concerning for MAS:
features were defined as any combination of the following:
?? Persistent (rather than quotidian) fever.
?? Cytopenias or falling cell line counts (particularly platelets).
?? Falling erythrocyte sedimentation rate.
?? Hypertriglyceridemia.
?? Hypofibrinogenemia.
?? Hemophagocytosis.
?? Transaminitis.
?? Coagulopathy.
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November 2013 | Rheumatology Forum