IL-1 Inhibition in Systemic Juvenile Idiopathic Arthritis

Giancane, Gabriella; Minoia, Francesca; Davì, Sergio; Bracciolini, Giulia; Consolaro, Alessandro; Ravelli, Angelo

doi:10.3389/fphar.2016.00467

Cited by 40 publications

(29 citation statements)

References 44 publications

(69 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Sixteen (88.9%) of our patients responded well to tocilizumab treatment. Furthermore, anakinra and canakinumab are successfully used in patients with resistant sJIA and MAS . We noted the clinical and laboratory response to treatment with anakinra in 20 (74%) patients.…”

Section: Discussionmentioning

confidence: 85%

Prognosis, complications and treatment response in systemic juvenile idiopathic arthritis patients: A single‐center experience

et al. 2019

View full text Add to dashboard Cite

Aim Systemic juvenile idiopathic arthritis (sJIA) is a distinctive subtype of JIA characterized by systemic features and poor outcome. We aimed to investigate demographic and clinical features, long‐term treatment response and disease complications in a large sJIA cohort. Methods Patients diagnosed with sJIA followed up at a pediatric rheumatology outpatient department from January 2003 to December 2017 were included. Demographic and clinical features, long‐term treatment response and disease complications were retrospectively collected. Results A total of 168 sJIA patients (51.8% female, 48.2% male) were included: 31.5% with monocyclic, 13.7% polycyclic and 54.8% with persistent clinical course. Corticosteroids were initially used in all patients. Methotrexate was used in 75% and cyclosporine A was used in 17.3% patients. Biological drugs were used in 42.8% patients; etanercept in 29.7%, anakinra in 16%, canakinumab in 16%, tocilizumab in 10% patients. Remission off medication was achieved in 82 (48.8%). Macrophage activation syndrome (MAS) was present in 11.9%, growth retardation in 11.3% patients. Eight percent (4/50) of patients had low bone mineral density. Three patients (1.78%) died due to MAS secondary multiorgan insufficiency and infection. Conclusion The disease is characterized with diverse clinical presentation and possibly severe complications. MAS complicated with multiorgan insufficiency is the major mortality factor. Corticosteroids represent the mainstay of the initial treatment. In patients resistant to classic treatment, biological drugs should be timely introduced.

show abstract

Section: Discussionmentioning

confidence: 85%

Prognosis, complications and treatment response in systemic juvenile idiopathic arthritis patients: A single‐center experience

et al. 2019

View full text Add to dashboard Cite

show abstract

“…Anakinra is a recombinant non‐glycosylated inhibitor of the IL‐1 receptor that blocks the actions of IL‐1. Initiation of Anakinra therapy early in the disease course may improve outcome; it can be used as first line therapy or as corticosteroid‐sparing regime . Canakinumab is a long‐acting, high‐affinity human monoclonal antibody that selectively binds to human IL‐1β.…”

Section: Il‐1 In Autoinflammatorydiseasesmentioning

confidence: 99%

“…Initiation of Anakinra therapy early in the disease course may improve outcome; it can be used as first line therapy or as corticosteroid-sparing regime. 84 Canakinumab is a long-acting, high-affinity human monoclonal antibody that selectively binds to human IL-1β. Canakinumab has demonstrated efficacy in randomized, placebo-controlled trials in active and febrile systemic juvenile idiopathic arthritis patients, and early steroid-free treatment with Canakinumab may lead to long-lasting remission.…”

Section: Il-1 In Autoinflammatory Diseasesmentioning

confidence: 99%

Interleukin 1 gene polymorphism and susceptibility to disease

Khazim

Azulay

Kristal

et al. 2017

Immunological Reviews

View full text Add to dashboard Cite

The Interleukin 1 (IL-1) family plays a central role in the generation and regulation of inflammatory responses, in both innate and adaptive immunity. Although the IL-1 molecules are traditionally considered to be classical proinflammatory cytokines, their functions are not restricted to inflammation, and they have also been shown to play a key role in a wide range of additional physiological and pathological functions, including learning modulation, sleep, pregnancy, depression, appetite, hematopoiesis, metabolism, and many others. Since their effect as cytokines and regulators of inflammation is so pleiotropic, any shift of the biological balance between agonistic and antagonistic signals has the potential to cause disease. Here, we consider the genetic influence of interleukin-1 gene polymorphism in the context of susceptibility to human diseases. We review known single nucleotide polymorphisms (SNP) of IL-1 genes linked to human diseases, and suggest how exploring biological effects of IL-1 gene cluster polymorphism may lead to new directions in understanding and diagnostic of disease and effective treatment.

show abstract

“…In SJIA, the three IL-1 blockers have been tested so far and were proven as effective and safe, although only canakinumab is currently approved for use (Giancane et al, 2016). In addition, anakinra has been demonstrated as efficient in severe SJIA with MAS (Miettunen et al, 2011) a severe complication that can occur in up to half of SJIA patients.…”

Section: Kawasaki Diseasementioning

confidence: 99%

IL-1 Inhibition May Have an Important Role in Treating Refractory Kawasaki Disease

Dusser

Koné‐Paut

2017

Front. Pharmacol.

View full text Add to dashboard Cite

Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10–20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need. Phenotypical and immunological similarities between KD and systemic juvenile idiopathic arthritis led to the hypothesis that KD could be considered as an autoinflammatory disease. New insights regarding KD’s pathogenesis have merged from the combination of genetic and transcriptomic data revealing the key role of interleukin-1 (IL-1) signaling in the pathogenesis of the vasculitis. Once activated, IL-1α and IL-1β trigger a local proinflammatory environment-inducing vasodilatation and attracting monocytes and neutrophils to sites causing tissue damage and stress. Both IL-1α and IL-1β have been shown to induce myocarditis and aneurysm formation in Lactobacillus casei cell-wall extract mouse model of KD; both being successfully improved with IL-1 blockade treatment such as anakinra. Treatment failure in patients with the high-risk inositol-triphosphate 3-kinase C genotype was associated with highest basal and stimulated intracellular calcium levels, increased cellular production of IL-1β, and IL-18, and higher circulating levels of both cytokines. Three clinical trials of IL-1 blockade enrolling KD patients are currently being conducted in Western Europe and in USA, they could change KD outcome.

show abstract

IL-1 Inhibition in Systemic Juvenile Idiopathic Arthritis

Cited by 40 publications

References 44 publications

Prognosis, complications and treatment response in systemic juvenile idiopathic arthritis patients: A single‐center experience

Prognosis, complications and treatment response in systemic juvenile idiopathic arthritis patients: A single‐center experience

Interleukin 1 gene polymorphism and susceptibility to disease

IL-1 Inhibition May Have an Important Role in Treating Refractory Kawasaki Disease

Contact Info

Product

Resources

About