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Ruling out mimics for systemic onset JIA often requires 'extensive workup'

Last updated: 08-11-2020

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Ruling out mimics for systemic onset JIA often requires 'extensive workup'

A quotidian fever lasting at least 2 weeks is one of the hallmarks of systemic onset juvenile idiopathic arthritis, according to a presenter at the 2020 Rheumatology Nurses Society Annual Conference.

“The common presentations for this condition are fever, arthritis and rash,” Carolyn Zic, MSN, FNP-BC, a pediatric rheumatology nurse at Comer Children’s Hospital at the University of Chicago Medicine, said in her presentation. She suggested that an “extensive workup” is often necessary to rule out infections, malignancies or even coronary disease that may look like systemic onset JIA. “The etiology is not currently known.”

Zic covered a broad range of topics pertaining systemic onset JIA in her presentation, from clinical manifestations to treatments, complications and considerations for the nursing community.

Most parents will first identify this condition based on the fever, according to Zic. She said that clinicians should be looking for a specific pattern fever. It spikes daily, usually in the evening, for at least 3 days and possibly up to 2 weeks.

However, Zic stressed that much more information is necessary to make this diagnosis. D-dimers are generally elevated, along with interleukin (IL)-1, IL-6, IL-8, TNF and macrophage migration inhibitory factor (MIF). Patients may exhibit elevations in a number of other laboratory parameters, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP), platelets, ferritin, fibrinogen, aldolase, ALT and AST. Anemia may also be present, along with low albumin.

In addition to the fever, patients should demonstrate at least one or more of the following: evanescent erythematous rash, generalized lymph node enlargement, hepatomegaly and/or splenomegaly or serositis. “The rash comes and goes,” Zic said.

There may also be musculoskeletal involvement, according to Zic. “When they do have arthritis, it is usually multiple joints,” she said.

Regarding cardiac and pulmonary manifestations, Zic said pericardial involvement is the most common. “You should do an echocardiogram and do a chest X-ray,” she said.

Turning to treatment paradigms, Zic highlighted data showing that canakinumab (Ilaris, Novartis), anakinra (Kineret, Sobi) and tocilizumab (Actemra, Genentech) demonstrated similar efficacy and safety in managing systemic onset JIA. That said, canakinumab may be more effective in broader systemic disease than in patients who have more severe arthritis.

Methotrexate and corticosteroids may also be included in treatment plans for systemic onset JIA, according to Zic. “Methotrexate does not help systemic features but is especially used for kids with a lot of arthritis,” she said.

Perhaps the most concerning feature that may accompany this disease is the potentially fatal macrophage activation syndrome (MAS). “There is no specific diagnostic biomarker for this,” Zic said. That said, IL-1 or IL-18 may be present, and researchers are currently investigating potential genetic components. Regardless of the reason for MAS, Zic was unequivocal in her recommendation for managing it. “You have to act quick.”

This is where nurses come into play. “We need to understand the acute or life-threatening complications of soJIA,” Zic said. “We should provide education for families about how to take the patient’s temperature, to know when to contact a provider and when to go to the emergency room.”

Nurses can also instruct patients and their families about the medications used to treat this disease, including the mechanisms of action and potential adverse events like injection site reactions.

Beyond the physical, nurses can also help patients and their families understand the psychosocial impacts of a chronic disease. “We can help parents or caregivers with FMLA paperwork or 504 plans, we can help them get support in school for children who need extra time to do their work,” Zic said. “We can also encourage the child to continue being active and eat healthy.”

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