Does systemic-onset juvenile idiopathic arthritis belong under juvenile idiopathic arthritis?

Abstract

'Science is the systematic classification of experience' George Henry Lewes (1817-78), English philosopher, critic, dramatist, scientist. Juvenile idiopathic arthritis (JIA) is prevalent in about 1 in 1000 children. The earliest formal description of this disease was by Sir George Frederick Still in 1897. This work was done when he was a registrar at the Hospital for Sick Children, Great Ormond Street, London. In this initial description of 19 patients he identified three patterns of arthritis, one of which came to be known later as Still's disease [now known as systemic-onset juvenile idiopathic arthritis (SoJIA)]. Over the next few decades it came to be appreciated that one form of arthritis in children is very different and dominated by the presence of systemic manifestations. Over the last two decades several paediatric rheumatologists have come together to classify juvenile arthritis for purposes of better disease identification and research. All along, the systemic form of juvenile arthritis was always recognized as belonging to a distinct group; in fact for several decades (and even now in some countries) the systemic form of juvenile arthritis was referred to as Still's disease. In this article we will attempt to highlight the reasons why we feel that SoJIA is perhaps not best retained in the company of JIA.

Effectiveness of First‐Line Treatment With Recombinant Interleukin‐1 Receptor Antagonist in Steroid‐Naive Patients With New‐Onset Systemic Juvenile Idiopathic Arthritis: Results of a Prospective Cohort Study

“…All patients were followed up for at least 12 months, with a mean followup after starting recombinant IL-1Ra of 32 months (range 12-54 months). The mean number of arthritic joints prior to the start of recombinant IL-1Ra treatment was 4.4 (range [1][2][3][4][5][6][7][8][9][10][11][12][13][14]. The clinical data, as well as laboratory or immunologic parameters of disease activity at the time that recombinant IL-1Ra was started, are shown in Table 1.…”

“…It has become clear that systemic JIA is an autoinflammatory disease rather than an autoimmune disease like the other subtypes of JIA (2)(3)(4)(5)(6). As such, the innate immune system seems to play a more prominent role in the pathophysiology of systemic JIA.…”

Relevance of application of the Yamaguchi criteria for patients with suspected juvenile idiopathic arthritis in the absence of arthritis symptoms

“…At onset, systemic JIA, which represent nearly 10% of all cases of JIA, is distinguished from other forms by prominence of extra-articular features such as spiking fever, typical fleeting pink macular rash, generalized lymphadenopathy, hepatosplenomegaly, and occasionally polyserositis [2]. The pathogenic mechanisms underlying the heterogeneity of systemic JIA are not well understood [3]. The aetiology of JIA is still unknown, and the genetic component is complex [4].…”

Current Pharmacotherapy for Systemic Juvenile Arthritis

“…Общая смертность при сЮА со-ответствует почти 2/3 всех случаев смерти среди детей с артритом, у 20% больных при непрерывно рецидивирующем течении сЮА к периоду достижения взрослого воз-раста развивается амилоидоз [5]. По мере на-копления новых сведений об особенностях иммунопатогенеза сЮА все более очевид-ным становится его принципиальное отли-чие от других ювенильных артритов, позво-ляющее рассматривать сЮА как аутовоспа-лительное заболевание [6,7], требующее принципиально иных подходов к терапии.…”