Treatment of Juvenile Spondyloarthritis: Where We Stand

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THERAPY IN PRACTICE

Treatment of Juvenile Spondyloarthritis: Where We Stand John M. Bridges1   · Matthew L. Stoll1 

© Springer Nature Switzerland AG 2020

Abstract Juvenile spondyloarthritis is a subset of juvenile idiopathic arthritis (JIA) with onset in late childhood and adolescence and a strong association with human leukocyte antigen (HLA) B-27 positivity and familial aggregation that has the potential for axial involvement, potentially leading to ankylosing spondylitis. Current therapy for severe disease relies heavily on tumor necrosis factor inhibitors (TNFi). Treatment paradigms in children largely consist of extrapolation from studies on adults with spondyloarthritis. Additional therapies studied in adults include non-steroidal anti-inflammatory drugs (NSAIDs), blockade of the interleukin-17 (IL-17) and IL-23 axes, blockade of T-cell stimulation, phosphodiesterase (PDE)-4 inhibition, and Janus-activated kinase (JAK) pathway alteration. IL-17 blockade and IL-23 blockade are guideline approved after TNFi failure (and even as an alternative to TNFi) in adults, depending on concomitant inflammatory bowel and skin disease, with JAK and PDE-4 inhibition options following biologic failure. Neither pediatric nor adult guidelines address IL-6 blockade, T-cell co-stimulation blockade, or combination biologic therapy.

Key Points  Tumor necrosis factor inhibitors are effective in children with spondyloarthritis. There is strong rationale for blockade of interleukin-17 (IL-17) or IL-23 in children with spondyloarthritis, but data are lacking. Much of our practice in the management of children with juvenile spondyloarthritis is based upon extrapolations from studies on adults.

1 Juvenile Spondyloarthritis (JSpA) Juvenile spondyloarthritis (JSpA) is a broad term used to describe arthritis that typically appears in late childhood and adolescence, with a proclivity for human leukocyte antigen (HLA) B-27 positivity and familial aggregation as well as * John M. Bridges [email protected] 1



Department of Pediatrics, University of Alabama at Birmingham, Birmingham, AL, USA

the potential for axial involvement. JSpA involves both the axial and peripheral skeleton. Axial involvement refers to inflammation of the sacroiliac joints and eventually the lumbar spine; the endpoint of axial inflammation, ankylosing spondylitis (AS), as defined by the modified New York criteria [1], is rare in children [2]. Peripheral spondyloarthritis (SpA) by definition involves non-axial joints, and often precedes the development of axial SpA [3]. Psoriatic juvenile idiopathic arthritis (PsJIA) has previously been defined by the International League of Associations for Rheumatology (ILAR), and the Pediatric Rheumatology International Trials Organization (PRINTO) proposed a new set of criteria for juvenile idiopathic arthritis (JIA), which places SpA patients into a category called enthesitis/spondylitis-related JIA [4, 5]. PsJIA is not included in this criteria set, an omission that has been criticized [6]. A comparison between the ILAR an