Abstract
Objective: We performed a single-center retrospective study to determine the different efficacy of tocilizumab (TCZ) in the early and late stages and in three phenotypic subgroups (monocyclic, polycyclic, and persistent) of systemic juvenile idiopathic arthritis (sJIA).Methods: Clinical and serological parameters of 77 sJIA patients treated by TCZ were collected from November 1, 2013 to May 1, 2019. Patients were grouped based on the duration group A < 6 months (n = 41) and group B > 6 months (n = 36) and divided into three phenotypes: monocyclic (n = 12), polycyclic (n = 14), and persistent (n = 51) course.Results: At baseline, group A had pronounced ESR, fever less active arthritis than group B (p < 0.05). After 12 weeks of therapy, TCZ alleviated fever, ESR, CRP, and systemic-onset juvenile arthritis disease activity score-27 (sJADAS27) in both group A and group B (p>0.05), while the efficacy of TCZ in relieving active arthritis in group A was better than that in group B (p<0.05). After 1 year of TCZ therapy, it showed that patients with monocyclic phenotype had the highest clinical response rate (91.7%, odds ratio = 0, 95% CI: 24–24, p = 0.00), followed by the polycyclic (28.6%, odds ratio = 2.1, 95% CI: 10.5–18.8, p = 0.00) and the persistent course (9.8%, odds ratio = 1.2, 95% CI: 9.5–13.8, p = 0.00).Conclusion: TCZ can quickly relieve fever and inflammation, especially when patients have less active arthritis with shorter disease duration. The long-term efficacy of TCZ is related to the phenotypes, among which the monocyclic is the best, and the persistent is the worst.
Highlights
Systemic juvenile idiopathic arthritis is a systemic inflammatory disease clinically characterized by fever, lymphadenopathy, arthritis, rash, and serositis. sJIA is the most serious subtype of juvenile idiopathic arthritis [1] and accounts for 30% to 40% of all JIA in Asia [2]
The enrolled patients who were in the active stage of the disease were allowed nonSteroidal anti-inflammatory drugs (NSAIDs), glucocorticoids, and disease-modifying antirheumatic drugs (DMARDs) (e.g., MTX, thalidomide, hydroxychloroquine, and leflunomide), among which the glucocorticoid dose was standardized to
Group A had pronounced erythrocyte sedimentation rate (ESR), fever, and less active arthritis compared to group B (p < 0.05)
Summary
Systemic juvenile idiopathic arthritis (sJIA) is a systemic inflammatory disease clinically characterized by fever, lymphadenopathy, arthritis, rash, and serositis. sJIA is the most serious subtype of juvenile idiopathic arthritis [1] and accounts for 30% to 40% of all JIA in Asia [2]. Systemic juvenile idiopathic arthritis (sJIA) is a systemic inflammatory disease clinically characterized by fever, lymphadenopathy, arthritis, rash, and serositis. SJIA is the most serious subtype of juvenile idiopathic arthritis [1] and accounts for 30% to 40% of all JIA in Asia [2]. A significant number of patients develop severe disease and treatment-related complications such as persistent arthritis, growth delay, and osteoporosis. Serious complications which are potentially fatal including macrophage activation syndrome (MAS) occur in 10% to 15% of children with sJIA [3,4,5]. The etiology of sJIA is not fully understood; proinflammatory cytokines including interleukin (IL)-6, IL-1, and IL-18 play an important role in the pathogenesis of the disease. Blockade of IL-6 represents the main mechanism of sJIA treatment and prevention of potential complications [11]
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