Study Evaluates Utility of Consensus Treatment Plans in First-Line Therapy for Systemic JIA

In a study presented at the American College of Rheumatology Convergence 2021, researchers sought to evaluate the efficacy and safety of Consensus Treatment Plans (CTPs) developed to standardize first-line treatment options for patients with systemic juvenile idiopathic arthritis (sJIA).

The four CTPs were developed by the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry and are defined as:

  • initial systemic glucocorticoid (GC) only
  • initial methotrexate (MTX) +/- GC
  • initial IL-1 inhibition (IL-1i) +/- GC
  • initial IL-6 inhibition (IL-6i) +/- GC

The researchers evaluated these CTPs in the prospective observational FROST study. Inclusion criteria included treatment initiation for recent onset sJIA, fever for at least two weeks, arthritis for at least days, and at least one of the following: evanescent rash, generalized lymphadenopathy, hepatomegaly, splenomegaly, or serositis. Patients were assigned treatments at the discretion of their physician and family. The primary endpoint was clinical inactive disease (CID; defined via Wallace ACR provisional criteria) and no current GC use after nine months of follow-up. Other secondary endpoints included CID regardless of current GC use and Clinical Juvenile Arthritis Disease Activity Score (cJADAS-10) without active fever.

Due to few patients being treated within the GC, MTX, and IL-6i arms, this analysis includes only the results for biologic therapy (IL-1i/IL-6i) and non-biologic therapy (GC/MTX) are presented. Intention to treat analysis was conducted.

Seventy-three patients were enrolled, comprising 63 patients who received biologic therapy and 10 patients who received non-biologic therapy. Fourteen percent of patients who initiated IL-1i were subsequently switched to IL-6i. No patients switched from IL-6i to IL-1i. Half of patients in the non-biologic arm eventually switched to biologic therapy within 101 days of initial treatment.

Nine-month follow-up data was available for 59 patients. The rate of CID without current GC use was 57% overall. Three-quarters of patients demonstrated a cJADAS-10 score of less than 2.5 with no fever or current GC use.

Overall, 16 grade ≥3 adverse events occurred, all of which were in the biologic therapy arm. Six episodes of Macrophage Activation Syndrome occurred and one patients died due to acute liver failure.

“Treatment practices have changed tremendously since the CARRA sJIA CTPs were initially developed,” the authors wrote. “Initial biologic therapy is now the most common treatment approach among sJIA patients enrolled in the CARRA Registry. A substantial proportion of patients not initially started on biologics subsequently started a biologic within the first few months following diagnosis. Excellent treatment outcomes were observed in 57% to 75% of FROST patients overall, depending on the outcome definition.”