Abstract

The juvenile spondyloarthropathies (JSpA) are a group of related rheumatic diseases characterized by involvement of peripheral large joints, axial joints, and entheses (enthesitis) that begin in the early years of life (prior to 16th birthday).The nomenclature and concept of spondyloarthropathies has changed during the last few decades. Although there is not any specific classification of JSpA, diseases under the spondyloarthropathy nomenclature umbrella in the younger patients include: the seronegative enthesitis and arthropathy (SEA) syndrome, juvenile ankylosing spondylitis, reactive arthritis, and inflammatory bowel disease-associated arthritis. Moreover, the ILAR criteria for Juvenile Idiopathic Arthritis includes two categories closely related to spondyloarthritis: Enthesitis-related arthritis and psoriatic arthritis.We review the pathophysiology and the use of biological agents in JSpA. JSpA are idiopathic inflammatory diseases driven by an altered balance in the proinflammatory cytokines. There is ample evidence on the role of tumor necrosis factor (TNF) and interleukin-17 in the physiopathology of these entities. Several non-biologic and biologic agents have been used with conflicting results in the treatment of these complex diseases. The efficacy and safety of anti-TNF agents, such as etanercept, infliximab and adalimumab, have been analysed in controlled and uncontrolled trials, usually showing satisfactory outcomes. Other biologic agents, such as abatacept, tocilizumab and rituximab, have been insufficiently studied and their role in the therapy of SpA is uncertain. Interleukin-17-blocking agents are promising alternatives for the treatment of JSpA patients in the near future. Recommendations for the treatment of patients with JSpA have recently been proposed and are discussed in the present review.

Highlights

  • The juvenile spondyloarthropathies (JSpA) are a group of related rheumatic diseases characterized by involvement of peripheral large joints, sacroiliitis / spondylitis and enthesitis that begin in the early years of life [1]

  • The results suggest that levels of synovial IL-17 in Enthesitis-related arthritis (ERA) correlate with disease activity, possibly due to locally induced MMP production by fibroblasts

  • The spondyloarthropathies are a diverse group of arthritides, classically involving large joints of the lower extremities, axial joints and entheses

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Summary

Background

The juvenile spondyloarthropathies (JSpA) are a group of related rheumatic diseases characterized by involvement of peripheral large joints, sacroiliitis / spondylitis and enthesitis that begin in the early years of life (prior to 16th birthday) [1]. Tse et al reported improvement in 10 children with JSpA on etanercept or infliximab followed for 1 year [38] In another retrospective study, 20 patients with JSpA showed good response to TNF inhibition (either etanercept (ETN), infliximab or adalimumab) when they had been refractory to NSAIDs [39]. In this study the proportion of patients who achieved ACR pediatric 30, 50, 70, 90, and 100 response rates at week 24 were 93, 93, 80, 56 and 54 % respectively during the initial open-label phase. Despite the association between increased serum IL-6 levels and disease activity in patients with AS reported in previous studies, IL-6 blockade did not correlate with clinical effectiveness in clinical practice. The efficacy and safety of Secukinumab (an anti-IL17A monoclonal antibody with proven efficacy in psoriasis), was tested in a double -blindtrial in adult patients with AS

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