Juvenile idiopathic arthritis- systemic

Disease ID:1176
Name:Juvenile idiopathic arthritis- systemic
Associated with:0 targets
0 immuno targets immuno relevant
5 immuno ligands
systemic juvenile rheumatoid arthritis
A subtype of juvenile idiopathic arthritis which affects the whole body, causing fever and rashes. Biologic drugs approved to treat systemic JIA include tocilizumab and canakinumab. This is a complex condition, with susceptibility influenced by many factors, including genetic polymorphisms.
Database Links
OMIM: 604302


No target related data available for Juvenile idiopathic arthritis- systemic


Ligand Approved Immuno References Clinical comments
Immuno Disease Comments: Approved therapy for systemic JIA.
canakinumab  [ FDA (2009), EMA (2009) (Drugs.com) ]
Clinical Use: Used to treat familial cold autoinflammatory syndrome (FCAS) and Muckle-Wells syndrome (MWS) in patients > 4 years of age, and systemic juvenile idiopathic arthritis (SJIA) in patients > 2 years old.
Immuno Disease Comments: An anti-IL-1β therapeutic approved for sJIA.
tocilizumab  [ FDA (2010), EMA (2009) (Drugs.com) ]
Clinical Use: Approved as a treatment for rheumatoid arthritis and systemic and polyarticular juvenile idiopathic arthritis.
In May 2017 tocilizumab became the first FDA approved drug for the treatment of adults with giant cell arteritis.
Phase 3 clinical trials for immune conditions including ankylosing spondylitis, hand osteoarthritis, systemic sclerosis and primary Sjögren's syndrome (pSS) are ongoing. Tocilizumab was initally used off-label to manage severe or life-threatening cytokine release syndrome (CRS), which is a serious, and potentially life-threatening side effect of CAR T-cell therapy. In September 2017, the FDA extended tocilizumab approval to include treatment of CAR T-cell therapy-induced CRS. It was approved particularly to manage CRS in patients ≥2 years of age receiving tisagenlecleucel (Kymriah®,CTL019), the first CAR T-cell therapy approved for relapsed and refractory B-cell ALL.
Immuno Disease Comments: An anti-IL-6 therapy approved for sJIA.
abatacept  [ FDA (2005), EMA (2005) (Drugs.com) ]
Clinical Use: Used to treat moderate to severe rheumatoid arthritis and juvenile rheumatoid arthritis. In July 2017, FDA approval was extended to include treatment of active psoriatic arthritis.
A Phase II clinical trial (NCT00505375) has been completed, evaluating the ability of abatacept to stop autoimmune destruction of any remaining active β cells in patients recently diagnosed with type 1 diabetes mellitus- see [3] for an explanation of the rationale behind this therapeutic approach, and [1] which points to some of its drawbacks.
Immuno Disease Comments: Approved drug for JIA.
deflazacort  [  (Drugs.com) ]
Clinical Use: Deflazacort can be prescribed for many inflammatory conditions including asthma, rheumatoid arthritis, Crohn's disease, juvenile chronic arthritis, idiopathic thrombocytopenic purpura, polymyalgia rheumatica, systemic lupus erythematosus and ulcerative colitis. More recently approved to treat Duchenne muscular dystrophy [2].
Immuno Disease Comments: Approved corticosteroid that can be prescribed for sJIA.


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1. Davis IC, Randell J, Davis SN. (2015) Immunotherapies currently in development for the treatment of type 1 diabetes. Expert Opin Investig Drugs24 (10): 1331-41. [PMID:26364507]

2. Griggs RC, Miller JP, Greenberg CR, Fehlings DL, Pestronk A, Mendell JR, Moxley 3rd RT, King W, Kissel JT, Cwik V et al.. (2016) Efficacy and safety of deflazacort vs prednisone and placebo for Duchenne muscular dystrophy. Neurology87 (20): 2123-2131. [PMID:27566742]

3. Ludvigsson J. (2016) Therapies to Preserve β-Cell Function in Type 1 Diabetes. Drugs76 (2): 169-85. [PMID:26645223]