ACR Juvenile Idiopathic Arthritis Guidelines Pocket Guide & App

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Juvenile Idiopathic Arthritis GUIDELINES Pocket Guide

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American College of Rheumatology

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Key Points

Juvenile arthritis is one of the most common chronic diseases of childhood, with an estimated prevalence of 1 per 1,000 children.
Following the exclusion of other known causes of synovitis, the term juvenile idiopathic arthritis (JIA) defines a heterogeneous collection of inflammatory arthritides of unknown etiology with onset prior to age 16 years and a minimum duration of 6 weeks.
All forms of JIA are associated with decreased health-related quality of life and risk of permanent joint damage. The disease may persist into adulthood, causing ongoing significant morbidity and impaired quality of life.
A number of treatments are available, including nonsteroidal antiinflammatory drugs (NSAIDs), systemic and intra-articular glucocorticoids, and nonbiologic and biologic disease-modifying antirheumatic drugs (DMARDs).
Prompt initiation of appropriate therapy is of critical importance in preventing permanent damage and improving outcomes.

Treatment

Grade Definitions on Strength of Recommendation and Guide to Interpretation

Strength		For the patient	For the clinician
*Strong*	“should use” or “should be used”	Most individuals in this situation would want the recommended course and only a small proportion would not.	Most individuals should receive the recommended course of action. Formal decision aids are not likely to be needed to help individuals make decisions consistent with their values and preferences.
Conditional	“may be used” or “may consider” or “Y (less preferred drug) may be used instead of X (preferred drug)” or “may consider Y instead of X (preferred drug)”	The majority of individuals in this situation would want the suggested course, but many would not.	Different choices would be appropriate for different patients. Decision aids may be useful in helping individuals in making decisions consistent with their values and preferences. Clinicians should expect to spend more time with patients when working towards a decision.

Grade Definitions of Quality and Certainty of the Evidence

Quality Grade	Definition
High	Very confident that the true effect lies close to that of the estimate of the effect.
Moderate	Moderately confident in the effect estimate. The true effect is likely to be close to the estimate of effect, but there is a possibility that it is substantially different.
Low	Confidence in the estimate is limited. The true effect may be substantially different from the estimate of effect.
Very low	Very little confidence in the effect estimate. The true effect is likely to be substantially different from the estimate of effect.

Table 1. Terms and definitions^a

Term	Definition
Polyarthritis population	Children with JIA and non-systemic polyarthritis (≥5 joints ever involved); may include children from ILAR JIA categories of polyarticular (rheumatoid factor positive or negative), extended oligoarticular, enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis.
Risk factors	One or more of the following: positive rheumatoid factor, positive anti-cyclic citrullinated peptide antibodies, joint damage.
Moderate/high disease activity	Clinical Juvenile Disease Activity Score based on the cJADAS-10 >2.5.
Low disease activity	Clinical JADAS-10 ≤2.5 and ≥1 active joint.
Sacroiliitis population	Patients with active sacroiliitis who will most likely be classified within the ILAR categories of enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis, but may include patients in any of the ILAR JIA categories.
Active sacroiliitis	Prior or current magnetic resonance imaging findings consistent with sacroiliitis along with clinical examination findings consistent with sacroiliitis (e.g., pain with direct palpation of the sacroiliac joints) and/or patient-reported symptoms of inflammatory back pain.
Enthesitis population	Patients with enthesitis (inflammation at tendon-to-bone insertion sites) who will most likely be from the ILAR categories of enthesitis-related arthritis, psoriatic arthritis, and undifferentiated arthritis, but may include patients from any of the ILAR JIA categories.
Active enthesitis	Tenderness and/or swelling of the entheses determined to require medical treatment per the treatment provider.

Table 2. Interventions included in the literature review

Intervention	Name/type
NSAIDs	Any
DMARDs	Leflunomide, methotrexate, sulfasalazine, triple non-biologic DMARD (methotrexate, sulfasalazine, hydroxychloroquine)
Biologics - TNFi	Adalimumab, etanercept, infliximab, golimumab^a
Biologics - Non-TNFi^b	Abatacept (CTLA-4Ig), tocilizumab (anti–interleukin-6 receptor), rituximab (anti-CD20)
Glucocorticoids - Oral	Any
Glucocorticoids - Intra-articular	Triamcinolone acetonide, triamcinolone hexacetonide, methylprednisolone acetate
Other interventions	Physical therapy, occupational therapy

^a Certolizumab was not included in the recommendations because no pediatric data were yet available.
^b Evaluated for polyarthritis only.

Table 3. General medication recommendations for children and adolescents with JIA and polyarthritis

Recommendation	Level of Evidence
Each recommendation is preceded by the phrase: “In children and adolescents with JIA and active polyarthritis…”
NSAIDs
NSAIDs are conditionally recommended as adjunct therapy.	Very low
DMARDs
Using methotrexate is conditionally recommended over leflunomide or sulfasalazine.	Moderate (leflunomide); very low (sulfasalazine)
Using subcutaneous methotrexate is conditionally recommended over oral methotrexate.	Very low
Glucocorticoids
Intra-articular glucocorticoids are conditionally recommended as adjunct therapy.	Very low
Triamcinolone hexacetonide is strongly recommended over triamcinolone acetonide for intra-articular glucocorticoid injections.	Moderate
Bridging therapy with a limited course of oral glucocorticoid (<3 months) during initiation or escalation of therapy in patients with high or moderate disease activity is conditionally recommended.^a Bridging therapy may be of most utility in the setting of limited mobility and/or significant symptoms.	Very low
Conditionally recommend against bridging therapy with a limited course of oral glucocorticoid (<3 months) in patients with low disease activity.	Very low
Strongly recommend against adding chronic low-dose glucocorticoid, irrespective of risk factors or disease activity.	Very low
Biologic DMARDs
In children and adolescents with JIA and polyarthritis, initiating treatment with a biologic (etanercept, adalimumab, golimumab, abatacept, or tocilizumab) combination therapy with a DMARD is conditionally recommended over biologic monotherapy.	Very low (etanercept, golimumab); low (abatacept, tocilizumab); moderate (adalimumab)
Combination therapy with a DMARD is strongly recommended for infliximab.	Low
Physical therapy and occupational therapy
In children and adolescents with JIA and polyarthritis who have or are at risk of functional limitations, using physical therapy and/or occupational therapy is conditionally recommended.	Low (physical therapy); very low (occupational therapy)

^a A bridging course of oral glucocorticoids was defined as a short course (<3 months) of oral glucocorticoids intended to control disease activity quickly during the initiation or escalation of therapy. An adequate trial of methotrexate was considered to be 3 months. If no or minimal response is observed after 6–8 weeks, it was agreed that changing or adding therapy may be appropriate.

Table 4. General guidelines for the initial and subsequent treatment of children and adolescents with JIA and polyarthritis^a

Recommendation^b	Level of Evidence
Each recommendation is preceded by the phrase: “In children and adolescents with JIA and active polyarthritis…”
Initial therapy
All patients
Initial therapy with a DMARD is *strongly* recommended over NSAID monotherapy.	Moderate
Using methotrexate monotherapy as initial therapy is conditionally recommended over triple DMARD therapy.	Low
Patients without risk factors:^b
Initial therapy with a DMARD is conditionally recommended over a biologic.	Low
Patients with risk factors:
Initial therapy with a DMARD is conditionally recommended over a biologic, recognizing that there are situations where initial therapy that includes a biologic may be preferred. Initial biologic therapy may be considered for patients with risk factors and involvement of high-risk joints (e.g., cervical spine, wrist, or hip), high disease activity, and/or those judged by their physician to be at high risk of disabling joint damage.	Low
Subsequent therapy: Low disease activity (cJADAS-10 ≤2.5 and ≥1 active joint)
For children receiving a DMARD and/or biologic:
Escalating therapy is conditionally recommended over no escalation of therapy. Escalation of therapy may include: Intra-articular glucocorticoid injection(s), optimization of DMARD dose, trial of methotrexate if not done, and adding or changing biologic.	Very Low
Subsequent therapy: Moderate/high disease activity (cJADAS-10 >2.5)
If patient is receiving DMARD monotherapy:
Adding a biologic to original DMARD is conditionally recommended over changing to a second DMARD.	Low
Adding a biologic is conditionally recommended over changing to triple DMARD therapy.	Low
If patient is receiving first TNFi (± DMARD):
Switching to a non-TNFi biologic (tocilizumab or abatacept) is conditionally recommended over switching to a second TNFi. A second TNFi may be appropriate for patients with good initial response to their first TNFi (i.e., secondary failure).	Very low
If patient is receiving second biologic:
Using TNFi, abatacept, or tocilizumab (depending on prior biologics received) is conditionally recommended over rituximab.	Very low

^a Disease activity (moderate/high and low) as defined by the clinical Juvenile Disease Activity Score based on 10 joints (cJADAS-10) is provided as a general parameter and should be interpreted within the clinical context.
^b Risk factors include the presence of any of the following: positive anti-cyclic citrullinated peptide antibodies, positive rheumatoid factor, or presence of joint damage. An adequate trial of methotrexate was considered to be 3 months. If no or minimal response is observed after 6–8 weeks, it was agreed that changing or adding therapy may be appropriate. For the purposes of these recommendations, triple DMARD therapy is methotrexate, sulfasalazine, and hydroxychloroquine. The term biologic refers to TNFi, abatacept, or tocilizumab for each of the recommendations, with the exception of the recommendation for patients with JIA and polyarthritis and moderate or high disease activity despite a second biologic, which includes rituximab. Shared decision-making between the physician, parents, and patient, including discussion of recommended treatments and potential alternatives, is recommended when initiating or escalating treatment.

Figure 1. Summary of primary recommendations for the initial and subsequent treatment of children with JIA and active polyarthritis

Table 5. Recommendations for the initial and subsequent treatment of children and adolescents with JIA and sacroiliitis

Recommendation	Level of Evidence
In children and adolescents with active sacroiliitis, treatment with an NSAID is strongly recommended over no treatment with an NSAID.	Very low
In children and adolescents with active sacroiliitis despite treatment with NSAIDs:
Adding TNFi is strongly recommended over continued NSAID monotherapy.	Low
Using sulfasalazine for patients who have contraindications to or have failed a TNFi is conditionally recommended.	Low
Strongly recommend against using methotrexate monotherapy.	Very low
Glucocorticoids
In children and adolescents with active sacroiliitis despite treatment with NSAIDs:
Bridging therapy with a limited course of oral glucocorticoid (<3 months) during initiation or escalation of therapy is conditionally recommended.^a Bridging therapy may be of most utility in the setting of high disease activity, limited mobility, and/or significant symptoms.	Very low
Intra-articular glucocorticoid injection of the sacroiliac joints as adjunct therapy is conditionally recommended.	Very low
Physical therapy
In children and adolescents with sacroiliitis who have or are at risk for functional limitations, using physical therapy is conditionally recommended.	Very low

Table 6. Recommendations for the initial and subsequent treatment of children and adolescents with JIA and enthesitis

Recommendation	Level of Evidence
In children and adolescents with active enthesitis, NSAID treatment is strongly recommended over no treatment with an NSAID.	Very low
In children and adolescents with active enthesitis despite treatment with NSAIDs:
Using a TNFi is conditionally recommended over methotrexate or sulfasalazine.	Low
Bridging therapy with a limited course of oral glucocorticoids (<3 months) during initiation or escalation of therapy is conditionally recommended.^a Bridging therapy may be of most utility in the setting of high disease activity, limited mobility, and/or significant symptoms.	Very low
Physical therapy
In children and adolescents with enthesitis who have or are at risk for functional limitations, using physical therapy is conditionally recommended.	Very low

^a A bridging course of oral glucocorticoids was defined a short course (<3 months) of oral glucocorticoids intended to control disease activity quickly during the initiation or escalation of therapy.

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Key Points

Treatment

Grade Definitions on Strength of Recommendation and Guide to Interpretation

Grade Definitions of Quality and Certainty of the Evidence

Table 1. Terms and definitionsa

Table 2. Interventions included in the literature review

Table 3. General medication recommendations for children and adolescents with JIA and polyarthritis

Table 4. General guidelines for the initial and subsequent treatment of children and adolescents with JIA and polyarthritisa

Figure 1. Summary of primary recommendations for the initial and subsequent treatment of children with JIA and active polyarthritis

Table 5. Recommendations for the initial and subsequent treatment of children and adolescents with JIA and sacroiliitis

Table 6. Recommendations for the initial and subsequent treatment of children and adolescents with JIA and enthesitis

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Table 1. Terms and definitions^a

Table 4. General guidelines for the initial and subsequent treatment of children and adolescents with JIA and polyarthritis^a