|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Proton pump inhibitors (PPIs), which are commonly used as inhibitors of gastric acid production, also have anti-inflammatory properties, protect mice from sepsis, and prevent IL-1β secretion by monocytes from patients with CAPS.
|
31194989 |
2020 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Cryopyrin-associated periodic syndrome (CAPS) is a prototypical autoinflammatory disease caused by gain-of-function mutations in the NLRP3 (NLR family pyrin domain containing 3) gene; these mutations activate the NLRP3 inflammasome, resulting in overproduction of IL-1β.
|
31185077 |
2019 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Gain-of-function mutations in NLRP3 in CAPS patients lead to activation of the cryopyrin inflammasome, resulting in the inappropriate release of inflammatory cytokines including IL-1β and CAPS-related inflammatory symptoms.
|
31077002 |
2019 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
The early intervention of IL-1β blockade may reduce the chance of complete deafness in patients with CAPS.
|
30338413 |
2019 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
In monocytes isolated from patients with cryopyrin-associated periodic syndrome (CAPS), OLT1177 inhibited LPS-induced IL-1β release by 84% and 36%.
|
29378952 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
IL-1 is a cytokine that exerts a pivotal role in innate immunity and in the pathogenesis of some autoimmune diseases, such as rheumatoid arthritis, and in autoinflammatory disorders, as familial Mediterranean fever and cryopyrin-associated periodic syndromes.
|
29633070 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Aberrant endochondral bone formation in the physis is a unique bone lesion in neonatal-onset multisystem inflammatory disease (NOMID), also called chronic infantile neurologic cutaneous articular (CINCA), the most severe of the cryopyrin-associated periodic syndrome (CAPS) diseases, which are interleukin-1β (IL-1β)-related monogenic autoinflammatory diseases.
|
29956067 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
In view of the critical role of IL-1ß in the pathogenesis of autoinflammatory GOF mutations such as CAPS, blockade of the action of IL-1ß is critical.
|
29610014 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Inflammasomes have also been involved in the pathogenesis of a wide range of autoinflammatory conditions that are caused by dysregulation of the IL-1 pathway, such as cryopyrinopathies and hereditary periodic fever syndromes.
|
29869008 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Consequently, we suggest that CAPS should be included in the differential diagnosis of adult patients with unexplained, recurrent inflammatory diseases, and once confirmed, the early initiation of anti-IL-1 blockade will probably prevent the development of life-threatening complications.
|
28079503 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
These rare conditions are collectively named "hereditary periodic fever syndromes" (HPFS), and protean pathogenetic mechanisms combined with several clinical phenotypes characterize at least four distinct conditions: (1) familial Mediterranean fever, which is the prototype and the most widely recognized among HPFS, inherited as an autosomal recessive disorder showing recurrent dysregulated inflammatory processes, caused by an abnormal interaction between cytoskeleton and inflammasome, a key-signaling platform that releases interleukin-1β (IL-1β); (2) the group of cryopyrin-associated periodic syndrome, which upsets directly the production of IL-1β, with a dominant pattern of inheritance; (3) tumor necrosis factor receptor-associated periodic syndrome, which is an autosomal dominant disorder subverting the functions and traffic of a cell membrane protein; and (4) mevalonate kinase deficiency, which is an autosomal recessive metabolic disorder halting the biosynthesis of cholesterol.
|
27068928 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Remarkably, single TLR4 stimulation is sufficient to activate massive, GSDMD-mediated IL-1β secretion in monocytes from patients affected by Cryopyrin Associated Periodic Syndrome (CAPS), an autoinflammatory disease linked to NLRP3 mutations.
|
30352992 |
2018 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Cryopyrin-associated periodic syndrome (CAPS) is a rare, heterogeneous disease entity associated with <i>NLRP3</i> gene mutations and increased interleukin-1 (IL-1) secretion.
|
27707729 |
2017 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Areas covered: Accounting for the pivotal role of IL-1ß in the pathogenesis of CAPS, three therapeutic options, all blocking the action of IL-1ß, are currently approved: anakinra, a recombinant IL-1 receptor antagonist, the IL-1 trap rilonacept and canakinumab, a monoclonal anti-IL-1ß antibody.
|
28586272 |
2017 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Cryopyrin-associated periodic syndromes (CAPS) result from gain-of-function mutations in the NLRP3 gene, which causes excessive release of interleukin-1β (IL-1β) and systemic inflammation.
|
28692792 |
2017 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
It specifically belongs to the cryopyrin-associated periodic syndromes (CAPSs) in which there is a mutation in the NLRP<sub>3</sub> (NLR family pyrin domain containing 3) gene that leads to overproduction of IL-1β, the source of the multisystem inflammatory symptoms.
|
28686751 |
2017 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Additionally, QUC inhibited IL-1β in Cryopyrin-Associated Periodic Syndromes (CAPS) macrophages, where NLRP3 inflammasome is constitutively activated.
|
28148962 |
2017 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Moreover, these results may have therapeutic implications for CAPS patients with partial responses to IL-1-targeted therapies.
|
29130929 |
2017 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
The pattern of secretion of IL-1β and other cytokines (IL-6 and IL-1 receptor antagonist) in patients did not display the aberrancies observed in patients with CAPS and was similar to that observed in healthy controls.
|
27036377 |
2016 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Cryopyrin-associated periodic syndromes (CAPS) are caused by mutations in the NLRP3 gene leading to overproduction of IL-1β and other NLRP3 inflammasome products.
|
27351923 |
2016 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
GeneticVariation
|
disease |
BEFREE |
Cryopyrin-associated periodic syndrome (CAPS) is a group of inherited autoinflammatory disorders caused by mutations in the NLRP3 gene resulting in the overproduction of interleukin 1β.
|
27134254 |
2016 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Remarkably, the oversecretion of IL-1β that represents a hallmark of monocytes from patients affected by cryopyrin-associated periodic syndrome is also blocked.
|
27441656 |
2016 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
IL-1 inhibitors block the IL-1 signaling cascade, thereby preventing systemic inflammation in CAPS.
|
26140469 |
2016 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
The objective of this study was to evaluate the efficacy and safety of treatment with the long-lasting fully humanised anti-IL-1β monoclonal antibody canakinumab in a Spanish cohort of patients with CAPS.
|
26243511 |
2016 |
|
Cryopyrin-Associated Periodic Syndromes
|
0.100 |
Biomarker
|
disease |
BEFREE |
Anti-IL-1 drugs have shown remarkable efficacy in treating CAPS symptoms and have significantly changed patients' lives.
|
26312542 |
2015 |