Dr. Becker also emphasized that when tapering these drugs, a high risk for flare exists. “Guidelines suggest preferentially using monoclonal antibody therapies over the use of etanercept because the benefit of using monoclonal antibody therapies has been shown,” she said.
MAS
The second JIA-associated complication Dr. Becker discussed was MAS. Developing in about 15% of patients with systemic JIA (sJIA; up to 30% as subclinical disease), MAS is characterized by a number of clinical features (e.g., high non-remitting fevers, hepatosplenomegaly, generalized lymphadenopathy, central nervous system dysfunction or encephalopathy, coagulopathy or hemorrhagic dysfunction) and has between an 8% and 20% mortality rate.
“Clearly, MAS is a significant risk for morbidity and mortality in children with sJIA,” said Dr. Becker. “Recognizing it quickly with clinical and lab features is imperative [as is] rapid institution of high-dose [glucocorticoids]. Cyclosporine and IL-1 [interleukin-1] blockade with high-dose anakinra (>4mg/kg/day) is often effective.”
Emerging data also suggest that treatments targeting various pathways—Janus kinase/signal transducer and activator of transcription, interferon-gamma and IL-18 binding protein—may also be effective to treat MAS in children. Dr. Becker discussed data showing that severe manifestations of sJIA, such as MAS, involve activation of interferon-γ mediated pathways and IL-18 overproduction. “These therapies are interesting and important in the emerging knowledge of the pathophysiology of MAS and the hyper-inflammatory state that is characteristic of MAS and distinct from sJIA,” she said.
Mary Beth Nierengarten is a freelance medical journalist based in Minneapolis.
References
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- Maccora I, Fusco E, Marrani E, et al. Changing evidence over time: Updated meta-analysis regarding anti-TNF efficacy in childhood chronic uveitis. Rheumatology (Oxford). 2021 Feb 1;60(2):588–587.
- Kahn P, Weiss M, Imundo LF, Levy DM. Favorable response to high-dose infliximab for refractory childhood uveitis. Ophthalmology. 2006 May;113(5):860–864.
