Abstract
BackgroundSystemic juvenile idiopathic arthritis (SoJIA) is the most striking form of juvenile idiopathic arthritis. The aim of our study was to evaluate the clinical responses and outcomes of children with SoJIA to IL-6 blockade using two different tocilizumab (TCZ) treatment protocols designed for milder and more severe SoJIA patient groups, and evaluate the possibility of achieving biologic-free remission.MethodsThirty-seven active SoJIA children who have failed treatment with corticosteroids and other DMARDs were included in our retrospective study. TCZ doses were prescribed in two treatment approaches: every 2 weeks TCZ dosing (Q2W) and every 4 weeks TCZ dosing (Q4W). The patients were assigned to these two groups by the study physicians depending on the severity of the SoJIA disease as judged by each clinician.ResultsThirty-three of the 37 children successfully completed the trial. TCZ was discontinued in 11patients during the trial. Seven children achieved inactive disease and were allowed to stop the TCZ and 4 had severe adverse events requiring drug cessation. Currently 7 patients continue to have TCZ-free remission [4/7 remission off-medication, 3/7still on methotrexate (MTX)]. This mixed group had a median treatment duration of 1002 days. The children in remission off of all medications, TCZ and MTX, had a median remission duration of 1162 days (ranged 932–1301 days).Compared to the patients assigned to the Q2W TCZ treatment group, the patients assigned to the Q4W TCZ group had a milder SoJIA course. The patients had higher levels of hemoglobin, total proteins, and serum albumins. They had lower white blood cell counts (WBC), % granulocytes, CRP, ESR, ferritins, and LDH. These children had a lower frequency of internal organ involvement, fewer relapses during TCZ treatment, and no macrophage activation syndrome episodes.ConclusionsOur experience with TCZ for SoJIA supports the excellent result of other studies. What may be novel is our finding that thisIL-6 blockade with TCZ may be able to be utilized at a less frequent dosing schedule in mild SoJIA compared to severe SoJIA. We discuss other factors that may increase the probability of a patient reaching TCZ-free remission.
Highlights
Systemic juvenile idiopathic arthritis (SoJIA) is the most striking form of juvenile idiopathic arthritis
The main demographic parameters included the median age (Me; Interquartile ranges (IQR)) of TCZ start of 10.2 years and the median delay from diagnosis to use of TCZ if 36.0 months
We summarized the provisional clinical signs and laboratory parameters before 1st TCZ infusion which may increase the future probability of reaching remission off TCZ (Table 4): no fever before TCZ initiation, no macrophage activation syndrome (MAS) after start of TCZ, no lymphadenopathy, absence of coagulopathy and CNS dysfunction and low disease activity, allowed to use TCZ every 4 weeks
Summary
Systemic juvenile idiopathic arthritis (SoJIA) is the most striking form of juvenile idiopathic arthritis. Systemic-onset juvenile idiopathic arthritis (SoJIA) is the most striking forms of juvenile idiopathic arthritis This challenging disease unchecked may lead to severe joint disability and internal organ involvement and is frequently associated with life-threatening complications such as macrophage activation syndrome and amyloidosis [1]. There are typical SoJIA-related long-term adverse events that have been noted for decades, both from the disease and the treatment with corticosteroids. These include anemia, Cushing’s syndrome, obesity, growth failure, osteoporosis with pathological fractures, aseptic bone necrosis, hypertension as well as metabolic disturbances such as hyperglycemia and dyslipidemia [2]. The IL-6 blocker is the only biologic drug available for SoJIA management at this time
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