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Update on the pathogenesis and treatment of systemic onset juvenile rheumatoid arthritis
Alexa Adams1, Thomas J A Lehman
1Division of Pediatric Rheumatology, Hospital for Special Surgery, Weill Cornell Medical Center, New York, NY 10021, USA.
Insights
New biologic therapies targeting specific cytokines are improving treatment for systemic onset juvenile rheumatoid arthritis. These advanced treatments offer better disease control with fewer side effects than traditional corticosteroid therapy.
Area of Science:
- Pediatric Rheumatology
- Immunology
- Molecular Medicine
Background:
- Systemic onset juvenile rheumatoid arthritis (SOJRA) presents significant challenges in pediatric care, often leading to severe physical and emotional disability.
- Understanding the complex etiopathogenesis of SOJRA is crucial for developing effective treatment strategies.
- Current therapeutic approaches aim to mitigate disease and treatment-related morbidities.
Purpose of the Study:
- To review recent advancements in understanding the etiopathogenesis of SOJRA.
- To highlight novel therapies targeting specific cytokines involved in SOJRA inflammation.
- To evaluate the efficacy and safety of new treatments compared to corticosteroids.
Main Methods:
- Review of recent scientific literature on SOJRA.
- Analysis of studies focusing on cytokine pathways and gene expression in SOJRA.
- Evaluation of clinical data for emerging biologic agents and other novel therapies.
Main Results:
- Advances in understanding cytokine dysregulation in SOJRA have enabled targeted therapies.
- Biologic agents targeting interleukin-1 (IL-1), interleukin-6 (IL-6), and tumor necrosis factor alpha (TNF-α) are in clinical use.
- Investigational agents targeting IL-18, myeloid-related proteins, NK cell function, and MIF are under study.
Conclusions:
- Anakinra (IL-1 inhibitor), anti-IL-6 receptor antibody, and thalidomide demonstrate significant clinical improvement in SOJRA.
- These novel therapies offer a superior side effect profile compared to traditional corticosteroid treatment.
- Targeted cytokine inhibition represents a promising therapeutic direction for managing SOJRA.
Purpose Of Review:
Although systemic onset juvenile rheumatoid arthritis accounts for only about 20% of most reported series, children with systemic onset juvenile rheumatoid arthritis are often the most difficult to treat. Many children with persistent systemic onset juvenile rheumatoid arthritis have marked physical and emotional disability as a result of both disease and treatment-related morbidities. This review highlights recent studies that better elucidate the etiopathogenesis of systemic onset juvenile rheumatoid arthritis. New therapies derived from better understanding of cytokines, cytokine gene expression, and their complex interactions, which result in inflammation, are improving our ability to control active disease while reducing or reliance on corticosteroids.
Recent Findings:
Recent advances in our understanding of the etiopathogenesis of systemic onset juvenile rheumatoid arthritis have led to therapies that specifically target the cytokines found in abnormal quantities in children with active disease. Biologic agents that directly target interleukin-1a, interleukin-6, and tumor necrosis factor alpha are currently in use, and additional agents that modulate interleukin-18, myeloid-related proteins 8 and 14, natural killer cell function, and macrophage migration inhibitory factor production are under investigation.
Summary:
Anakinra, monoclonal antibody to interleukin-6 receptor, and thalidomide each have led to significant clinical improvement with fewer side effects than resulted when corticosteroids were the mainstay of therapy.
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