Systemic Juvenile Rheumatoid Arthritis

A type of arthritis that occurs among some patients affected by juvenile chronic, or idiopathic, arthritis and characterized by severe extraarticular symptoms that include a spiking fever with a corresponding increase in the serum levels of INTERLEUKIN-6, UVEITIS; LYMPHADENOPATHY; HEPATOSPLENOMEGALY; SEROSITIS and MYALGIA. Polymorphisms in the IL6 and MIF genes are associated with susceptibility to this disorder. OMIM: 604302

Also Known As:

Networked: 42 relevant articles (9 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Arthritis (Polyarthritis)
2.	Castleman Disease (Castleman's Disease)
3.	Rheumatoid Arthritis
4.	Fever (Fevers)
5.	Exanthema (Rash)

Experts

1.	Kishimoto, Tadamitsu: 4 articles (04/2014 - 11/2006)
2.	Tanaka, Toshio: 2 articles (04/2014 - 02/2014)
3.	Cron, Randy Q: 1 article (06/2020)
4.	Hartwell, James: 1 article (06/2020)
5.	Manson, Jessica J: 1 article (06/2020)
6.	Mehta, Puja: 1 article (06/2020)
7.	Tattersall, Rachel S: 1 article (06/2020)
8.	Dasgupta, Bhaskar: 1 article (01/2017)
9.	Hadavi, Shahryar: 1 article (01/2017)
10.	Laskou, Faidra: 1 article (01/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Systemic Juvenile Rheumatoid Arthritis:

1.	Interleukin-6 (Interleukin 6)IBA 02/01/1998 - "Is systemic juvenile rheumatoid arthritis an interleukin 6 mediated disease?" 07/01/1992 - "Relationship between interleukin-6 levels and platelet counts in systemic juvenile rheumatoid arthritis: comment on the article by De Benedetti et al." 02/15/1997 - "Systemic juvenile rheumatoid arthritis is a chronic inflammatory disorder characterized by markedly elevated circulating levels of IL-6 and stunted growth. " 09/01/1991 - "Correlation of serum interleukin-6 levels with joint involvement and thrombocytosis in systemic juvenile rheumatoid arthritis." 05/01/2010 - "On the basis of the findings, a new therapeutic approach to block the actions of IL-6 by use of a humanized anti-IL-6R antibody has been proven to be therapeutically effective for rheumatoid arthritis, systemic juvenile idiopathic arthritis and Castleman's disease. "
2.	tocilizumab (atlizumab)FDA Link 02/01/2014 - "Worldwide clinical trials have demonstrated the outstanding efficacy of tocilizumab in rheumatoid arthritis, systemic juvenile idiopathic arthritis, and Castleman's disease; thus, a new era has come for the treatment of these diseases, which were previously considered intractable. " 01/01/2017 - "Tocilizumab (TCZ), is a recombinant humanized anti-interleukin-6 receptor (IL-6R) monoclonal antibody which has a main use in the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis (sJIA) and polyarticular juvenile idiopathic arthritis (pJIA). " 04/01/2014 - "Clinical trials using the humanized anti-IL-6 receptor monoclonal antibody tocilizumab have demonstrated the efficacy of IL-6 blockade for the treatment of refractory inflammatory diseases, such as rheumatoid arthritis, systemic juvenile idiopathic arthritis, and Castleman disease. " 11/01/2006 - "Blocking IL-6 actions by use of a humanized antibody, tocilizumab, which targets the IL-6 receptor, has been proven to be therapeutically effective for rheumatoid arthritis, systemic juvenile idiopathic arthritis, Castleman disease and Crohn's disease. "
3.	Interleukin-6 Receptors (Interleukin 6 Receptor)IBA 05/01/1994 - "By using a sandwich ELISA, soluble human IL-6 receptor (sIL-6 R) levels were measured in the sera of 20 healthy children and of 25 patients with systemic juvenile rheumatoid arthritis (JRA). " 05/01/1994 - "Serum soluble interleukin 6 (IL-6) receptor and IL-6/soluble IL-6 receptor complex in systemic juvenile rheumatoid arthritis." 09/01/1999 - "Decreased IL-10 production, an anti-inflammatory cytokine, and soluble IL-6 receptor are associated with systemic juvenile rheumatoid arthritis (JRA). " 10/01/2013 - "In clinical studies, IL-6 and IL-6 receptor antibodies have proven efficacy in rheumatoid arthritis, systemic juvenile idiopathic arthritis, and Castleman's disease, conditions that are known to be driven by IL-6. " 11/01/2006 - "Blocking IL-6 actions by use of a humanized antibody, tocilizumab, which targets the IL-6 receptor, has been proven to be therapeutically effective for rheumatoid arthritis, systemic juvenile idiopathic arthritis, Castleman disease and Crohn's disease. "
4.	Intravenous Immunoglobulins (IVIG)FDA Link 01/01/1998 - "The study was based on the follow-up of 20 patients with systemic juvenile rheumatoid arthritis who received intravenous immunoglobulin. " 12/01/1994 - "Intravenous immunoglobulin in the treatment of systemic juvenile rheumatoid arthritis: a randomized placebo controlled trial. " 11/01/1995 - "In a similar fashion, intravenous immune globulin infusions were very effective in ameliorating systemic manifestations of systemic juvenile rheumatoid arthritis but less effective in controlling long-lasting arthritis (more than 1 year). " 11/01/1995 - "We have used monthly infusions of intravenous immune globulin (2 g/kg/month) in patients with dermatomyositis or systemic juvenile rheumatoid arthritis who have not responded to traditional treatment. " 10/01/1989 - "Pilot studies in dermatomyositis and polymyositis and systemic juvenile rheumatoid arthritis indicate benefit from intravenous immune globulin therapy. "
5.	Methotrexate (Mexate)FDA LinkGeneric 02/01/1996 - "A macrophage activation syndrome, possibly related to methotrexate toxicity, developed in a boy with systemic juvenile rheumatoid arthritis. " 10/01/1990 - "Treatment with methotrexate effectively controlled fever and rash in 83% of children with systemic juvenile rheumatoid arthritis, reduced morning stiffness by 63%, eliminated recalcitrant joint restriction in 48%, and reduced numbers of swollen joints and swelling indexes by 46% and 52%, respectively. " 04/01/1992 - "Repetitive use of pulse therapy with methylprednisolone and cyclophosphamide in addition to oral methotrexate in children with systemic juvenile rheumatoid arthritis--preliminary results of a longterm study."
6.	gamma-Globulins (gamma-Globulin)IBA 07/01/1990 - "In view of the successful treatment of these diseases, we initiated a pilot study of the effect of IV gamma globulin in systemic juvenile rheumatoid arthritis (JRA). " 04/01/1993 - "[Immunomodulating therapy of systemic juvenile rheumatoid arthritis: administration of high-dose intravenous gamma globulin]." 07/01/1990 - "Intravenous gamma globulin therapy in systemic juvenile rheumatoid arthritis." 06/01/1991 - "Clinical trials provided further data needed to assess the efficacy of high-dose intravenous gamma-globulin therapy in patients with systemic juvenile rheumatoid arthritis, and extended uncontrolled experience with interferon-gamma in adult rheumatoid arthritis was obtained. "
7.	Interferon-gamma (Interferon, gamma)IBA 05/01/1989 - "Nine severely ill patients with a confirmed diagnosis of systemic juvenile rheumatoid arthritis were treated with recombinant gamma-interferon (gamma-IFN) in addition to the therapy they were previously receiving for their disease. " 05/01/1989 - "Therapy for systemic juvenile rheumatoid arthritis with gamma-interferon: a pilot study of nine patients." 06/01/1991 - "Clinical trials provided further data needed to assess the efficacy of high-dose intravenous gamma-globulin therapy in patients with systemic juvenile rheumatoid arthritis, and extended uncontrolled experience with interferon-gamma in adult rheumatoid arthritis was obtained. "
8.	Monoclonal AntibodiesIBA 01/01/2017 - "Tocilizumab (TCZ), is a recombinant humanized anti-interleukin-6 receptor (IL-6R) monoclonal antibody which has a main use in the treatment of rheumatoid arthritis, systemic juvenile idiopathic arthritis (sJIA) and polyarticular juvenile idiopathic arthritis (pJIA). " 04/01/2014 - "Clinical trials using the humanized anti-IL-6 receptor monoclonal antibody tocilizumab have demonstrated the efficacy of IL-6 blockade for the treatment of refractory inflammatory diseases, such as rheumatoid arthritis, systemic juvenile idiopathic arthritis, and Castleman disease. "
9.	AntibodiesIBA 10/01/2013 - "In clinical studies, IL-6 and IL-6 receptor antibodies have proven efficacy in rheumatoid arthritis, systemic juvenile idiopathic arthritis, and Castleman's disease, conditions that are known to be driven by IL-6. "
10.	Cyclosporine (Ciclosporin)FDA LinkGeneric 01/01/1999 - "To develop an effective and safe therapeutic policy for Sandimmun-Neoral in order to prevent joint destruction, invalidation, achieve higher life quality in patients with systemic juvenile rheumatoid arthritis (SJRA). " 01/01/1999 - "[Sandimmun-Neoral--a new quality of life for patients with severe systemic juvenile rheumatoid arthritis]." 01/01/1988 - "Cyclosporin treatment of a patient with severe systemic juvenile rheumatoid arthritis." 02/01/1996 - "Macrophage activation syndrome in systemic juvenile rheumatoid arthritis successfully treated with cyclosporine."

Therapies and Procedures

1.	Therapeutics 05/01/1996 - "[Nonconventional therapy in a case of systemic juvenile rheumatoid arthritis]." 11/01/1995 - "Similarly, some patients with systemic juvenile rheumatoid arthritis continue to develop crippling arthritis despite the currently used modalities of therapy. " 01/01/2003 - "The aims of this study were: (1) to assess functional status, emotional well-being and quality of life in patients with polyarticular and systemic juvenile rheumatoid arthritis (JRA) treated with etanercept, and (2) to determine the prevalence and significance of adverse events associated with etanercept therapy. " 01/01/2001 - "The authors studied the response to and side effects of long-term glucocorticoid (GC) therapy of systemic juvenile rheumatoid arthritis (SJRA). " 04/01/1993 - "[Immunomodulating therapy of systemic juvenile rheumatoid arthritis: administration of high-dose intravenous gamma globulin]."
2.	Pericardiectomy 04/01/1984 - "Cardiac tamponade in systemic juvenile rheumatoid arthritis requiring emergency pericardiectomy."
3.	Subcutaneous Injections 06/01/2020 - "Anakinra, a recombinant humanised IL-1 receptor antagonist, is licenced at a dose of 100 mg once daily by subcutaneous injection for rheumatoid arthritis, systemic juvenile idiopathic arthritis, adult-onset Still's disease, and cryopyrin-associated periodic syndromes. "