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Juvenile idiopathic arthritis: Old disease, new tactics

The Journal of Family Practice. 2019 March;68(2):E8-E13
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Beyond NSAIDs and disease-modifying antirheumatic drugs are now biologic agents and anti-interleukin drugs that can augment therapy.

PRACTICE RECOMMENDATIONS

› Pair the findings of your clinical exam with the results of imaging and laboratory testing to make the diagnosis of juvenile idiopathic arthritis (JIA), as it is a diagnosis of exclusion. B

› Individualize treatment based on where the patient falls in the JIA disease spectrum to increase the likelihood that medical therapy will be effective. A

› Consider treating diagnosed JIA with an available biologic agent, which can provide a long asymptomatic period. B

Strength of recommendation (SOR)

A Good-quality patient-oriented evidence
B Inconsistent or limited-quality patient-oriented evidence
C Consensus, usual practice, opinion, disease-oriented evidence, case series

For example, there is an increased frequency of autoimmune diseases among JIA patients.18 There are also reports documenting an increased rate of infection, including with enteric pathogens, parvovirus B,19 rubella, mumps, hepatitis B, Epstein-Barr virus, mycoplasma, and chlamydia.19 Stress and trauma have also been implicated.12

The T-lymphocyte percentage is increased in the synovial fluid of JIA patients, although that percentage varies from subtype to subtype.20 This elevation results in an increase in the number of macrophages, which are induced by secreted cytokines to produce interleukin (IL)-1, IL-6, and tumor necrosis factor alpha (TNF-a). This activity of cellular immunity leads to joint destruction.21

Clinical features

The most common signs and symptoms of JIA are arthralgias (39%), arthritis (25%), fever (18%), limping (9%), rash (8%), abdominal pain (1.3%), and uveitis (1.3%).15 Forty percent of JIA patients are reported to have temporomandibular joint involvement at some point in their life; mandibular asymmetry secondary to condylar resorption and remodeling17 is the most common presenting complaint—not arthralgia or pain, as would be expected.

Most JIA patients (52%) first present to the emergency department; another 42% present to the office of a general medical practitioner.15 On average, 3 visits to a physician, over the course of approximately 3 months, are made before a definitive diagnosis (usually by a pediatric rheumatologist) is made.15

Pertinent questions to ask a patient who has a confirmed diagnosis of JIA include the nature, severity, and duration of morning stiffness and pain, as well as any encumbering factors to regular functioning at home or school.22 Different scoring charts can be used to determine the extent of pain and disability, including the Juvenile Arthritis Disease Activity Score (JADAS)23 and the clinical JADAS (cJADAS),24 which measure minimal disease activity25 and clinically inactive disease26 cutoffs.

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