Randy Q. Cron, MD, PhD, prepared useful practice aids pertaining to juvenile arthritis for this CME/MOC/CNE activity titled "Systemic Juvenile Idiopathic Arthritis: A Closer Look at the Burden on Patients and Caregivers From Diagnosis to Treatment." For the full presentation, monograph, complete CME/MOC/CNE information, and to apply for credit, please visit us at http://bit.ly/2rtHUur. CME/MOC/CNE credit will be available until December 26, 2019.

1. This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
Systemic Juvenile Idiopathic Arthritis (sJIA)
Classification Criteria
a
The criteria had a sensitivity of 0.73 and a specificity of 0.99.
1. Petty RE et al. J Rheumatol. 2004;31:390-392. 2. Ravelli A et al. Ann Rheum Dis. 2016;75:481-489.
PRACTICE AID
Access the activity, “Systemic Juvenile Idiopathic Arthritis: A Closer Look at the Burden on
Patients and Caregivers From Diagnosis to Treatment,” at www.peerview.com/AVC40.
Ferritin
>684 ng/mL
• Platelet counts ≤181 x 109
/L
• Aspartate aminotransferase
>48 units/L
• Triglycerides >156 mg/dL
• Fibrinogen ≤360 mg/dL
Plus any
two of
Arthritis in ≥1 joint with or
preceded by ≥2 weeks of fever
that is quotidian for ≥3 days,
plus ≥1 of the following:
• Evanescent (nonfixed)
erythematous rash
• Serositis
• Hepatomegaly and/or splenomegaly
• Generalized lymphadenopathy
Macrophage Activation Syndrome in sJIA Classification Criteria2,a
International League of Associations for Rheumatology sJIA Classification Criteria1

2. This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
Therapeutic Agents Commonly Used for
Systemic Juvenile Idiopathic Arthritis (sJIA)1
CNS: central nervous system; MAS: macrophage activation syndrome; subQ: subcutaneous.
a
Dose varies based on NSAID choice.
1. Grevich S, Shenoi S. Adolesc Health Med Ther. 2017;8:125-135.
PRACTICE AID
Access the activity, “Systemic Juvenile Idiopathic Arthritis: A Closer Look at the Burden on
Patients and Caregivers From Diagnosis to Treatment,” at www.peerview.com/AVC40.
NSAIDa
Dose/Route Other CommentsMedication
• Naproxyn
• Indomethacin
10-20 mg/kg/day divided twice daily, orally
Maximum dose: 1,000 mg/day
1.5-3 mg/kg/day divided 2-3 times daily, orally
Maximum dose: 150 mg/day
Use for mild disease or during initial evaluation
while excluding other causes of fever
Cortico-
steroid
• Prednisone
• Pulse
methylprednisolone
1-2 mg/kg/day orally
Maximum dose: 60 mg/day
30 mg/kg/day for 3 days IV
Maximum dose: 1 g/day
Use if MAS or severe sJIA with
serious organ involvement, such as
pericarditis, myocarditis, pulmonary,
or CNS involvement
• Anakinra
• Canakinumab
1-4 mg/kg/day subQ or IV
≥2 years: 4 mg/kg dose every 4 weeks subQ
Maximum dose: 300 mg
Dose has been used in 10-15 mg/kg/day IV
for severe sJIA or MAS (half-life 4-6 hours)
Higher doses and frequencies have been
used to obtain adequate control of sJIA
(half-life at ≥4 years of age, 23-26 days)
Anti–IL-1
Therapy
• Tocilizumab
<30 kg, 12 mg/kg dose every 2 weeks
≥30 kg, 8 mg/kg dose every 2 weeks IV fusion
Maximum dose: 800 mg
Half-life is 1 week
Anti–IL-6
Therapy
Other
Agents
• Methotrexate
• Cyclosporine
0.5-1 mg/kg weekly or 10-15 mg/m2
weekly,
orally, IV, or subQ
Maximum dose: 25 mg/week
3-5 mg/kg divided twice daily, orally, or IV
Preference may be subQ administration; use
when arthritis dominates the clinical picture;
oral absorption may decrease, especially
at higher doses
Can use in MAS; oral absorption achieves
60% of level of IV dosing

3. Access the activity, “Systemic Juvenile Idiopathic Arthritis: A Closer Look
at the Burden on Patients and Caregivers From Diagnosis to Treatment,”
at www.peerview.com/AVC40.
Understanding the Burden of
Systemic Juvenile Idiopathic
Arthritis (sJIA)1
PRACTICE AID
This Practice Aid has been provided as a quick reference to help learners apply the information to their daily practice and care of patients.
1. https://acrabstracts.org/abstract/the-disease-burden-of-systemic-juvenile-idiopathic-arthritis-for-patients-and-caregivers-an-international-health-related-quality-of-life-survey-and-
retrospective-chart-review/. Accessed December 4, 2018.
The Burden of sJIA
Up to 45% of caregivers for children with sJIA
experience emotional distress caused by worry about
the long-term impact of the disease on their child
Caregivers of children with sJIA experience
impaired mental well-being
Over one-third of caregivers of children with sJIA who were
surveyed either stopped working or reduced their hours
of employment because of their child’s condition
Children with sJIA suffer significant impairment to their
quality of life, in terms of physical and mental well-being
25% of children with sJIA, who were surveyed, experience
difficulty with day-to-day activities,
such as gripping objects or reaching for a coat
Within a surveyed group, children with sJIA lost
an average of 3 school days
because of their condition the previous 2 months
45%
25%