The Systemic Juvenile Idiopathic Arthritis Cohort of the Childhood Arthritis and Rheumatology Research Alliance Registry: 2010–2013

Abstract

Most patients in the largest sJIA cohort reported to date had low disease activity. Practice patterns for choice of biologic agents appeared to change over the study period. Nearly one-third had persistent arthritis without systemic symptoms > 2 years after onset. African Americans were associated with worse disease activity. Strategies are needed to improve outcomes in subgroups with poor prognosis.

Genetic architecture distinguishes systemic juvenile idiopathic arthritis from other forms of juvenile idiopathic arthritis: clinical and therapeutic implications

“…Similarly, therapies targeting the cytokine tumour necrosis factor-α are highly effective in the treatment of other forms of JIA,22 but show only modest effect in children with sJIA 10. Today, even with the most effective treatments for sJIA directed against the inflammatory cytokines interleukin (IL)-1 and IL-6,10 a sizable proportion of children continue to have active disease, with chronic arthritis persisting in nearly 40% of children in a recent study 9. Currently, the only widely effective treatment for sJIA remains large doses of glucorticoids 10.…”

“…This has been challenged by identification of autoantibodies in some patients with sJIA 8. Furthermore, while the systemic inflammatory features of sJIA seem to distinguish it from other forms of JIA, most children with sJIA eventually shed these features, leaving up to half of children with a persistent form of arthritis that is similar to the oligoarticular and polyarticular forms of JIA 5 9. Finally, significant differential effects of anticytokine agents have been observed between sJIA and other forms of JIA 10.…”

Pilot study comparing the Childhood Arthritis & Rheumatology Research Alliance (CARRA) systemic Juvenile Idiopathic Arthritis Consensus Treatment Plans

“…The quandary about the safest and most effective treatment of new-onset systemic JIA has been informed by studies of the IL1i anakinra (recombinant IL-1 receptor antagonist). Recent studies looking at early use of anakinra in systemic JIA demonstrated a marked reduction in the development of chronic persistent arthritis from a historical rate of 50–60% to less than 10% [9, 11], a rate of response markedly distinct from that observed in a randomized controlled trial of anakinra in established chronic systemic JIA [2]. These studies raise the possibility of a “window of opportunity” in early systemic JIA during which cytokine blockade could be especially effective [23].…”

“…Funding from NIAMS via the American Recovery and Reinvestment Act (ARRA) in 2010 enabled the CARRA Registry, a multi-center prospective observational study of children with pediatric rheumatic diseases including JIA. Altogether, investigators enrolled over 9,500 patients in what is now called the CARRA Legacy Registry, including more than 7,000 with JIA (>500 with systemic JIA) at 60 sites over 3 years [9]. The CARRA Registry general protocol and consent was approved by the Duke University IRB (#Pro00054616) and at all participating site IRBs.…”

Síndrome febril prolongado de origen reumatológico. Reporte de tres casos.

“…A pesar de lo descrito, el paciente con SFP, adulto o pediátrico, continúa siendo un reto diagnóstico para el médico en las diferentes áreas, sea por la heterogeneidad clínica y paraclínica a través del tiempo o por la necesidad de instaurar un tratamiento efectivo (5,6). A continuación se presentan tres casos clínicos con SFP como manifestación de enfermedades reumatológicas en diferentes grupos etarios.…”