scholarly journals POS1294 EFFECT OF EMOTIONAL TRIGGERS ON THE FREQUENCY OF PFAPA ATTACKS - A MULTICENTER STUDY

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 928-929
Author(s):  
Y. Levinsky ◽  
Y. Butbul ◽  
M. Gafner ◽  
M. Broide ◽  
S. Kagan ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Emotional Distress ◽  
Effortful Control ◽  
Periodic Fever ◽  
Aphthous Stomatitis ◽  
Stressful Event ◽  
P Value ◽  
Cervical Adenitis ◽  
Mediterranean Fever ◽  
Distress Scale

Background:The fact that mental state may serve as a trigger for attacks of periodic fever diseases is known in Familial Mediterranean fever (FMF). There is a lack of information regarding the role of emotional triggers for PFAPA attacks.Objectives:To examine whether emotional distress may trigger PFAPA attacks.Methods:Enrolled were patients with active PFAPA, from two Israeli medical centers. Researchers contacted their parents by phone in two occasions: a. within the two weeks of returning to school after the first COCID-19 lockdown; b. during the summer vacation (i.e., non-stressful period). Parents were asked regarding the occurrence of PFAPA attacks within the two weeks preceding the calls. The relative stress levels of the two periods were validated by an emotional distress scale questionnaire. Differences between occurrences of attacks during these two periods of time were recorded.Results:One-hundred and six pediatric patients enrolled in the study. Mean age was 7.37± 2.9. In the stressful period, 41 (38.7%) reported at least one attack during the preceding 2 weeks, compared to 24 (22.6%) in the non-stressful period (p = 0.017). Scores for the mean emotional distress questionnaire were higher in the stressful period compared to the non-stressful period (35.6± 8.1 vs. 32.1± 7.7, respectively, P = 0.047).Conclusion:This study shows that during a period of stress for children there were more PFAPA attacks compared to a relaxed period. These findings indicate that mental factors such as stress or excitement may be triggers for PFAPA attacks.Table 1.Attacks status during prior 2 weeks during stressful or non-stressful eventOutcomeFirst call(May 2020)Second call (August 2020)P-valuePediatric emotional distress score (PEDS), mean (±SD)35.6 (±8.1)32.1 (±7.7)0.047Any attacks during last 2 week, n (%)41 (39.8%)24 (24.2%)0.017Number of attacks0.04 One, n (%)27 (25.5%)19 (17.9%) Two, n (%)14 (13.2%)5 (4.7%)References:[1]Marshall GS, Edwards KM, Butler J, Lawton AR. Syndrome of periodic fever, pharyngitis, and aphthous stomatitis. J Pediatr. 1987;110(1):43-46. doi:10.1016/S0022-3476(87)80285-8[2]Feder HM, Salazar JC. A clinical review of 105 patients with PFAPA (a periodic fever syndrome). Acta Paediatr Int J Paediatr. 2010;99(2):178-184. doi:10.1111/j.1651-2227.2009.01554.x[3]Hall, J, Lindorff A. Children’s Transition to School: Relationships Between Preschool Attendance, Cortisol Patterns, and Effortful Control. The Educational and Developmental Psychologist. Educ Dev Psychol. 2017;34(1):1-18[4]Stokes SJ, Saylor CF, Swenson CC, Daugherty TK. A comparison of children’s behaviors following three types of stressors. Child Psychiatry Hum Dev. 1995;26(2):113-123. doi:10.1007/BF02353235[5]Førsvoll J, Kristoffersen EK, Øymar K. The immunology of the periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis syndrome; what can the tonsils reveal. A literature review. Int J Pediatr Otorhinolaryngol. 2020;130. doi:10.1016/j.ijporl.2019.109795[6]Gidron Y, Berkovitch M, Press J. Psychosocial correlates of incidence of attacks in children with Familial Mediterranean Fever. J Behav Med. 2003;26(2):95-104. doi:10.1023/A:1023038504481[7]Makay B, Emiroğlu N, Ünsal E. Depression and anxiety in children and adolescents with familial Mediterranean fever. Clin Rheumatol. 2010;29(4):375-379. doi:10.1007/s10067-009-1330-9[8]Kraszewska-Głomba B, Matkowska-Kocjan A, Szenborn L. The Pathogenesis of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis Syndrome: A Review of Current Research. Mediators Inflamm 2015;2015:563876. doi:10.1155/2015/563876Disclosure of Interests:None declared

scholarly journals Periodic fever, aphthous stomatitis, pharyngitis, and adenitis syndrome, Hodgkin's lymphoma,and the familial Mediterranean fever gene

2014 ◽  
Vol 1 (1) ◽  
pp. 37
Author(s):  
Ala Atamna ◽  
Corina Hershkovici ◽  
Avishay Elis
Keyword(s):  
Familial Mediterranean Fever ◽  
Hodgkin’S Lymphoma ◽  
Periodic Fever ◽  
Gene Mutations ◽  
Aphthous Stomatitis ◽  
High Rate ◽  
Hodgkin's Lymphoma ◽  
Inflammatory Disorder ◽  
Mediterranean Fever ◽  
Pfapa Syndrome

The syndrome of periodic fever, aphthous stomatitis, pharyngitis, and adenopathy (PFAPA) is a benign, self-limited, auto-inflammatory disorder. The attacks usually start at age 2 to 5 years and cease before the age of 10 years. Affected children grow and develop normally and experience no long-term sequelae. The course of PFAPA syndrome is attenuated by the carrier state of the familial Mediterranean fever (FMF) gene, MEFV. A high rate of MEFV mutations has also been reported in various hemato-lymphoid neoplasms. We describe a 19-year-old woman with PFAPA syndrome in remission who was diagnosed with Hodgkin's lymphoma. Although mutations in the FMF gene were not found in this case, the association of PFAPA syndrome with FMF gene mutations and hematological malignancies is discussed.

Familial Mediterranean Fever Is Commonly Diagnosed in Children in Israel with Periodic Fever Aphthous Stomatitis, Pharyngitis, and Adenitis Syndrome

2019 ◽  
Vol 204 ◽  
pp. 270-274 ◽  
Author(s):  
Yonatan Butbul Aviel ◽  
Liora Harel ◽  
Maryam Abu Rumi ◽  
Riva Brik ◽  
Nofar Hezkelo ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Periodic Fever ◽  
Aphthous Stomatitis ◽  
Mediterranean Fever

The Familial Mediterranean Fever Gene as a Modifier of Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Adenopathy Syndrome

2011 ◽  
Vol 40 (5) ◽  
pp. 467-472 ◽  
Author(s):  
Yackov Berkun ◽  
Ran Levy ◽  
Adam Hurwitz ◽  
Michal Meir-Harel ◽  
Merav Lidar ◽  
...  
Keyword(s):  
Familial Mediterranean Fever ◽  
Periodic Fever ◽  
Aphthous Stomatitis ◽  
Mediterranean Fever

AB0980 CASE-REVIEW ON FAMILIAL MEDITERRANEAN FEVER IN A SPECIALIZED CENTRE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1784.1-1784
Author(s):  
R. Dos Santos Sobrín ◽  
M. Martí Masanet ◽  
B. Lopez-Montesinos ◽  
L. Lacruz Pérez ◽  
I. Calvo
Keyword(s):  
Juvenile Idiopathic Arthritis ◽  
Familial Mediterranean Fever ◽  
Periodic Fever ◽  
Severe Disease ◽  
Strong Association ◽  
Mefv Gene ◽  
Systemic Juvenile Idiopathic Arthritis ◽  
Case Series ◽  
Case Review ◽  
Mediterranean Fever

Background:Familial Mediterranean Fever (FMF) is a genetic autoinflammatory disorder caused mostly by mutations in MEFV gene. Its inheritance is autosomal recessive and is the most frequent periodic fever syndrome. First-line treatment is based in colchicine use, so biologics (anti-IL-1) are reserved for refractory cases1, 2.Objectives:To account for clinic and treatment features of patients with FMF in a specialized center as opposed to non-referent centers.Methods:This study was developed in the Pediatric Rheumatology Service in Hospital Universitario y Politécnico La Fe de Valencia. Demographic, clinic and treatment data were collected from patients diagnosed of FMF since January 2004 to September 2019.Results:106 patients met last FMF criteria3. 55% had a pathogenic mutation in genetic analysis. 52% were female. Before 10 years old, 71% of patients had the diagnosis (51% before 4 years old). Arthralgia/myalgia (73%), periodic fever (62%) and abdominal pain (54%) were the most common symptoms. Systemic Juvenile Idiopathic Arthritis (JIA, 6), other forms of JIA (9) and vasculitis (10) were the most prevalent comorbidities. When talking about treatment, 76,4% received Colchicine (60,5% with good response), 22,6% needed a classical disease modifying antirheumatic drug (mostly Methotrexate) and 22 patients got biologic treatment (73% anti-IL-1).Conclusion:When analyzing this case-review, JIA has a strong association with our patients, so it could explain severe disease activity and more articular involvement. This could be an illustration to the higher use of Methotrexate. Also, the most relevant symptom was arthralgia while fever is the most frequent in literature. Likewise, age of diagnosis has been earlier than other case-series (this would be more frequent in other autoinflammatory syndromes, as literature relates)1, 2, 4.References:[1]Ozdogan H, Ugurlu S. Familial Mediterranean Fever. Presse Med. (2019).[2]Ozen S, Demirkaya E, Erer B, et al. EULAR recommendations for the management of familial Mediterranean fever. Ann Rheum Dis 2016;75:644-651.[3]Sag E, Demirel D, Demir S, et al. Performance of the new “Eurofever/PRINTO classification criteria” in FMF patients. Semin Arthritis Rheum. 2019;19:30369-5.[4]Rozenbaum M, Rosner I. Severe outcome of juvenile idiopathic arthritis (JIA) associated with familial Mediterranean fever (FMF). Clin Exp Rheumatol. 2004;22:S75-8.Disclosure of Interests:Raquel Dos Santos Sobrín: None declared, Miguel Martí Masanet: None declared, B Lopez-Montesinos: None declared, Lucía Lacruz Pérez: None declared, Inmaculada Calvo Grant/research support from: Bristol-Myers Squibb, Clementia, GlaxoSmithKline, Hoffman-La Roche, Merck Sharpe & Dohme, Novartis, Pfizer, Sanofi, Speakers bureau: AbbVie, GlaxoSmithKline, Hoffman-La Roche, Novartis

scholarly journals THU0019 INTRON REGIONS OF PIK3AP1 (BCAP) AND SPON2 (SPONDIN-2) GENES ARE DIFFERENTIALLY METHYLATED IN PATIENTS WITH PERIODIC FEVER, APHTHOUS STOMATITIS, PHARYNGITIS AND ADENITIS (PFAPA) SYNDROME

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 223.3-223
Author(s):  
E. Lovšin ◽  
J. Kovac ◽  
T. Tesovnik ◽  
N. Toplak ◽  
D. Perko ◽  
...  
Keyword(s):  
Dna Methylation ◽  
Pattern Recognition ◽  
Periodic Fever ◽  
Aphthous Stomatitis ◽  
Extracellular Matrix Protein ◽  
Inflammasome Activation ◽  
Cervical Adenitis ◽  
Pfapa Syndrome ◽  
Pattern Recognition Molecule ◽  
Recognition Molecule

Background:Periodic fever, aphthous stomatitis, pharyngitis and adenitis (PFAPA) syndrome is the most common autoinflammatory disease in children, often grouped together with hereditary periodic fever syndromes, although its cause and hereditary nature remain unexplained.Objectives:We investigated whether a differential DNA methylation was present in DNA from peripheral blood mononuclear cells (PBMC) in patients with PFAPA versus a group of healthy young individuals.Methods:A whole epigenome analysis (MeDIP and MBD) was performed using pooled DNA libraries enriched for methylated genomic regions. Of identified candidate genes, two with most significantly different methylation leves were further evaluated with methylation specific restriction enzymes coupled with qPCR (MSRE-qPCR).Results:The analysis showed thatPIK3AP1andSPON2intronic gene regions are differentially methylated in patients with PFAPA. MSRE-qPCR proved as a quick, reliable and cost-effective method to confirm results from MeDIP and MBD.Conclusion:Our findings indicate that B cell adapter protein (BCAP) as PI3K binding inhibitor of inflammation and spondin-2 (SPON2) as a pattern recognition molecule and integrin ligand could play a role in etiology of PFAPA. Their role and impact of changed DNA methylation in PFAPA etiology and autoinflammation need further investigation.References:[1]Wekell P. Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis syndrome – PFAPA syndrome. Press Medicale [Internet]. 2019;48(1):e77–87. Available from:https://doi.org/10.1016/j.lpm.2018.08.016[2]K. Theodoropoulou, F. Vanoni, and M. Hofer, “Periodic Fever, Aphthous Stomatitis, Pharyngitis, and Cervical Adenitis (PFAPA) Syndrome: a Review of the Pathogenesis,”Curr. Rheumatol. Rep., vol. 18:18, 2016.[3]Carpentier SJ, Ni M, Duggan JM, James RG, Cookson BT, Hamerman JA. The signaling adaptor BCAP inhibits NLRP3 and NLRC4 inflammasome activation in macrophages through interactions with Flightless-1. Sci Signal. 2019;12(581).[4]He YW, Li H, Zhang J, Hsu CL, Lin E, Zhang N, et al. The extracellular matrix protein mindin is a pattern-recognition molecule for microbial pathogens. Nat Immunol. 2004;5(1):88–97.Disclosure of Interests:None declared

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