PReS-FINAL-2194: Evidence-based clinical classification criteria for periodic fevers

Federici, Silvia; Özen, Seza; Koné‐Paut, Isabelle; Lachmann, Helen; Woo, P; Cantarini, Luca; Amaryan, Gayane; Insalaco, Antonella; Kuemmerle‐Deschner, J. B.; Neven, Bénédicte; Dewarrat, Natacha; Uziel, Yosef; Rigante, Donato; Herlin, Troels; Martino, Silvana; Simon, A; Stojanov, S; Özdoğan, Huri; Frenkel, Joost; Ruperto, Nicolino; Martini, Alberto; Sormani, MP; Hofer, Michaël; Gattorno, Marco

doi:10.1186/1546-0096-11-s2-o29

Cited by 2 publications

(7 citation statements)

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“…40%) similarly occurred in all patients[12,18]. In CAPS patients, 5% vs. 3%), abdominal pain (37.5% vs. 18%), and chest pain/pericarditis (37.5% vs. 4%)[18]. The only patient with HIDS in our study presented with relatively well tolerated episodes of fever, cutaneous rash, headache, arthralgia, odynophagia, hepatomegaly and enlarged cervical 220 lymph nodes.…”

supporting

confidence: 51%

“…40%) similarly occurred in all patients[12,18]. In CAPS patients, 5% vs. 3%), abdominal pain (37.5% vs. 18%), and chest pain/pericarditis (37.5% vs. 4%)[18].…”

mentioning

confidence: 84%

“…Similarly to pediatric patients with PFAPA, all our adult patients had fever and odynophagia (tonsillitis and/or 223 pharyngitis). Enlarged cervical lymph nodes were also found in a similar proportion (60% vs. 70%)[18]. However, only 40% of them presented with aphtous stomatitis, compared with 68% of pediatric patients[18,20].…”

mentioning

confidence: 95%

“…The only patient with HIDS in our study presented with relatively well tolerated episodes of fever, cutaneous rash, headache, arthralgia, odynophagia, hepatomegaly and enlarged cervical 220 lymph nodes. All these manifestations are frequently presented by pediatric MKD patients[18,19]. Similarly to pediatric patients with PFAPA, all our adult patients had fever and odynophagia (tonsillitis and/or 223 pharyngitis).…”

mentioning

confidence: 95%

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Clinical and genetic characterization of the autoinflammatory diseases diagnosed in an adult reference center

Hernández‐Rodríguez

Ruiz-Ortiz

Tomé

et al. 2016

Autoimmunity Reviews

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supporting

confidence: 51%

“…40%) similarly occurred in all patients[12,18]. In CAPS patients, 5% vs. 3%), abdominal pain (37.5% vs. 18%), and chest pain/pericarditis (37.5% vs. 4%)[18].…”

mentioning

confidence: 84%

mentioning

confidence: 95%

mentioning

confidence: 95%

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Clinical and genetic characterization of the autoinflammatory diseases diagnosed in an adult reference center

Hernández‐Rodríguez

Ruiz-Ortiz

Tomé

et al. 2016

Autoimmunity Reviews

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“…One such disease is TNF Receptor Associated Periodic Syndrome (TRAPS) caused by mutations in TNF receptor-1 (TNFR1) [8], resulting in spontaneous episodes of systemic inflammation causing morbidity and potential mortality [9][10][11]. Current treatment options for TRAPS include corticosteroids (with potentially major side-effects) and cytokine-blocking biologics; although the latter represent a major advance in the management of TRAPS, they are expensive, may require specialist administration, and show variable, unpredictable, limited efficacy [12,13].…”

Section: Introductionmentioning

confidence: 99%

A signalome screening approach in the autoinflammatory disease TNF receptor associated periodic syndrome (TRAPS) highlights the anti-inflammatory properties of drugs for repurposing

Todd

Negm

Reps

et al. 2017

Pharmacological Research

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2Abbreviations: BCA, bicinchoninic acid; CV, coefficient of variation; MTT, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide; PBMCs, peripheral blood mononuclear cells; RPPA, reverse phase protein array; SSMD, strictly standardised mean difference; TNFR1, tumour necrosis factor receptor-1; TRAPS, TNF receptor associated periodic syndrome; WT, wild type. 3ABSTRACT: TNF Receptor Associated Periodic Syndrome (TRAPS) is an autoinflammatory disease caused by mutations in TNF Receptor 1 (TNFR1). Current therapies for TRAPS are limited and do not target the pro-inflammatory signalling pathways that are central to the disease mechanism. Our aim was to identify drugs for repurposing as anti-inflammatories based on their ability to down-regulate molecules associated with inflammatory signalling pathways that are activated in TRAPS. This was achieved using rigorously optimised, high through-put cell culture and reverse phase protein microarray systems to screen compounds for their effects on the TRAPS-associated inflammatory signalome. 1360 approved, publically available, pharmacologically active substances were investigated for their effects on 40 signalling molecules associated with pro-inflammatory signalling pathways that are constitutively upregulated in TRAPS. The drugs were screened at four ten-fold concentrations on cell lines expressing both wild-type (WT) TNFR1 and TRAPS-associated C33Y mutant TNFR1, or WT TNFR1 alone; signalling molecule levels were then determined in cell lysates by the reversephase protein microarray. A novel mathematical methodology was developed to rank the compounds for their ability to reduce the expression of signalling molecules in the C33Y-TNFR1 transfectants towards the level seen in the WT-TNFR1 transfectants. Seven high-ranking drugs were selected and tested by RPPA for effects on the same 40 signalling molecules in lysates of peripheral blood mononuclear cells (PBMCs) from C33Y-TRAPS patients compared to PBMCs from normal controls. The fluoroquinolone antibiotic lomefloxacin, as well as others from this class of compounds, showed the most significant effects on multiple pro-inflammatory signalling pathways that are constitutively activated in TRAPS; lomefloxacin dose-dependently significantly reduced expression of 7/40 signalling molecules across the Jak/Stat, MAPK, NF-kB and PI3K/AKT pathways. This study demonstrates the power of signalome screening for identifying candidates for drug repurposing.

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PReS-FINAL-2194: Evidence-based clinical classification criteria for periodic fevers

Cited by 2 publications

References 0 publications

Clinical and genetic characterization of the autoinflammatory diseases diagnosed in an adult reference center

Clinical and genetic characterization of the autoinflammatory diseases diagnosed in an adult reference center

A signalome screening approach in the autoinflammatory disease TNF receptor associated periodic syndrome (TRAPS) highlights the anti-inflammatory properties of drugs for repurposing

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