{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T02:43:44Z","timestamp":1785293024876,"version":"3.55.0"},"reference-count":47,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2022,4,27]],"date-time":"2022-04-27T00:00:00Z","timestamp":1651017600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Pediatr."],"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To evaluate the number of episodes in the past 12 months as an indicator of the overall disease activity status in Familial Mediterranean fever (FMF).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this cross-sectional study, patients were recruited from tertiary pediatric hospitals. Demographic data, main clinical symptoms of the episodes, treatment modalities, and genetic mutations were recorded. The patients were grouped as no episodes (Group 1), 1\u20134 episodes (Group 2), and more than 4 episodes (Group 3) according to the number of episodes in the past 12 months. The Pediatric Quality Life Inventory (PedsQL), the Children\u2019s Depression Inventory (CDI), and the Wong-Baker FACES Pain Rating Scale (FACES) scores were compared between groups. Concurrent validity between the number of episodes and the patient-reported outcome measures (PROMs) was assessed using Spearman\u2019s rank correlation coefficient (\u03c1).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 239 patients were included. There were 74 patients (31%) in Group 1, 99 (41.4%) in Group 2, and 66 (27.6%) in Group 3. Groups were similar according to age, age at diagnosis, gender, consanguinity, family history, history of amyloidosis, clinical symptoms, and in terms of allele frequency (<jats:italic>p<\/jats:italic> &amp;gt; 0.05). According to PROMs completed by parents, moderate correlations were found between the number of episodes and the PedsQL score (\u03c1 = \u22120.48; 95% <jats:italic>CI<\/jats:italic> = \u22120.58 to \u22120.35, <jats:italic>p<\/jats:italic> &amp;lt; 0.001) and between the number of episodes and the Wong-Baker FACES score (\u03c1 = 0.47, 95% <jats:italic>CI<\/jats:italic> = 0.35\u20130.57, <jats:italic>p<\/jats:italic> &amp;lt; 0.001).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The number of episodes was positively and moderately correlated with patient- and parent-reported outcomes in our cohort. The number of episodes in patients with FMF can be used as a single measure to assess disease activity.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fped.2022.822473","type":"journal-article","created":{"date-parts":[[2022,4,27]],"date-time":"2022-04-27T06:24:35Z","timestamp":1651040675000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Number of Episodes Can Be Used as a Disease Activity Measure in Familial Mediterranean Fever"],"prefix":"10.3389","volume":"10","author":[{"given":"David","family":"Piskin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zehra Serap","family":"Arici","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dilek","family":"Konukbay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Micol","family":"Romano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Balahan","family":"Makay","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nuray","family":"Ayaz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yelda","family":"Bilginer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Roberta A.","family":"Berard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hakan","family":"Poyrazoglu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ozgur","family":"Kasapcopur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald M.","family":"Laxer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathy","family":"Speechley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Erkan","family":"Demirkaya","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2022,4,27]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","first-page":"662","DOI":"10.1136\/annrheumdis-2012-202708","article-title":"Results from a multicentre international registry of familial Mediterranean fever: impact of environment on the expression of a monogenic disease in children.","volume":"73","author":"Ozen","year":"2014","journal-title":"Ann Rheum Dis."},{"key":"B2","doi-asserted-by":"publisher","first-page":"1636","DOI":"10.1136\/annrheumdis-2015-207546","article-title":"Recommendations for the management of autoinflammatory diseases.","volume":"74","author":"ter Haar","year":"2015","journal-title":"Ann Rheum Dis."},{"key":"B3","doi-asserted-by":"publisher","first-page":"644","DOI":"10.1136\/annrheumdis-2015-208690","article-title":"EULAR recommendations for the management of familial Mediterranean fever.","volume":"75","author":"Ozen","year":"2016","journal-title":"Ann Rheum Dis."},{"key":"B4","doi-asserted-by":"publisher","first-page":"1051","DOI":"10.1136\/annrheumdis-2015-208671","article-title":"Development and initial validation of international severity scoring system for familial Mediterranean fever (ISSF).","volume":"75","author":"Demirkaya","year":"2016","journal-title":"Ann Rheum Dis."},{"key":"B5","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1007\/s11926-015-0558-4","article-title":"Current Research in Outcome Measures for Pediatric Rheumatic and Autoinflammatory Diseases.","volume":"18","author":"Demirkaya","year":"2016","journal-title":"Curr Rheumatol Rep."},{"key":"B6","first-page":"129","article-title":"A novel assessment tool for clinical care of patients with autoinflammatory disease: juvenile autoinflammatory disease multidimensional assessment report.","volume":"34","author":"Konukbay","year":"2016","journal-title":"Clin Exp Rheumatol."},{"key":"B7","doi-asserted-by":"publisher","first-page":"309","DOI":"10.1136\/ard.2010.132613","article-title":"A preliminary score for the assessment of disease activity in hereditary recurrent fevers: results from the AIDAI (Auto-Inflammatory Diseases Activity Index) Consensus Conference.","volume":"70","author":"Piram","year":"2011","journal-title":"Ann Rheum Dis."},{"key":"B8","doi-asserted-by":"publisher","first-page":"2168","DOI":"10.1136\/annrheumdis-2013-203666","article-title":"Validation of the auto-inflammatory diseases activity index (AIDAI) for hereditary recurrent fever syndromes.","volume":"73","author":"Piram","year":"2014","journal-title":"Ann Rheum Dis."},{"key":"B9","doi-asserted-by":"publisher","first-page":"1908","DOI":"10.1056\/NEJMoa1706314","article-title":"Canakinumab for the Treatment of Autoinflammatory Recurrent Fever Syndromes.","volume":"378","author":"De Benedetti","year":"2018","journal-title":"N Engl J Med."},{"key":"B10","doi-asserted-by":"publisher","first-page":"395","DOI":"10.1093\/rheumatology\/ken509","article-title":"A new set of criteria for the diagnosis of familial Mediterranean fever in childhood.","volume":"48","author":"Yalcinkaya","year":"2009","journal-title":"Rheumatology."},{"key":"B11","doi-asserted-by":"publisher","first-page":"154","DOI":"10.3899\/jrheum.141249","article-title":"Performance of different diagnostic criteria for familial mediterranean fever in children with periodic fevers: results from a multicenter international registry.","volume":"43","author":"Demirkaya","year":"2016","journal-title":"J Rheumatol."},{"key":"B12","doi-asserted-by":"publisher","first-page":"909","DOI":"10.1089\/gtmb.2011.0046","article-title":"The use of a reverse hybridization strip assay for the study of hemochromatosis-associated gene mutations in Lebanon.","volume":"15","author":"Daher","year":"2011","journal-title":"Gen Testing Mol Biomark."},{"key":"B13","doi-asserted-by":"publisher","first-page":"126","DOI":"10.1097\/00005650-199902000-00003","article-title":"The PedsQL: measurement model for the pediatric quality of life inventory.","volume":"37","author":"Varni","year":"1999","journal-title":"Med Care."},{"key":"B14","first-page":"353","article-title":"\u00c7ocuklar i\u00e7in ya\u015fam kalitesi \u00f6l\u00e7e\u011finin 13-18 ya\u015f ergen formunun ge\u00e7erlik ve g\u00fcvenilirli\u011fi.","volume":"18","author":"Memik","year":"2007","journal-title":"T\u00fcrk Psikiyatri Dergisi."},{"key":"B15","author":"\u00dcneri","year":"2005","journal-title":"\u00c7ocuklar i\u00e7in Ya\u015fam Kalitesi \u00d6l\u00e7e\u011finin 2-7 ya\u015flar\u0131ndaki T\u00fcrk \u00e7ocuklar\u0131nda ge\u00e7erlik ve g\u00fcvenirli\u011fi."},{"key":"B16","first-page":"305","article-title":"Rating scales to assess depression in school-aged children.","volume":"46","author":"Kovacs","year":"1981","journal-title":"Acta Paedopsychiatr Int J Child Adolesc Psychiat."},{"key":"B17","first-page":"137","article-title":"Cocuklar icin Depresyon Olcegi gecerlik ve guvenirlik calismasi.","volume":"2","author":"Oy","year":"1991","journal-title":"Turk Psikiyatri Dergisi."},{"key":"B18","doi-asserted-by":"publisher","first-page":"11","DOI":"10.1097\/00006416-198701000-00003","article-title":"QUEST: a process of pain assessment in children.","volume":"6","author":"Baker","year":"1987","journal-title":"Orthop Nurs."},{"key":"B19","author":"Dancey","year":"2007","journal-title":"Statistics Without Maths for Psychology."},{"key":"B20","doi-asserted-by":"publisher","first-page":"393","DOI":"10.1002\/art.24477","article-title":"Disease control and health-related quality of life in juvenile idiopathic arthritis.","volume":"61","author":"Seid","year":"2009","journal-title":"Arthritis Rheum."},{"key":"B21","doi-asserted-by":"publisher","first-page":"1330","DOI":"10.3899\/jrheum.081028","article-title":"Health-related quality of life, physical function, fatigue, and disease activity in children with established polyarticular juvenile idiopathic arthritis.","volume":"36","author":"Ringold","year":"2009","journal-title":"J Rheumatol."},{"key":"B22","doi-asserted-by":"publisher","first-page":"1536","DOI":"10.3899\/jrheum.081164","article-title":"Health-related quality of life and its relationship to patient disease course in childhood-onset systemic lupus erythematosus.","volume":"36","author":"Brunner","year":"2009","journal-title":"J Rheumatol."},{"key":"B23","doi-asserted-by":"publisher","first-page":"2051","DOI":"10.1093\/rheumatology\/ker256","article-title":"Sleep and fatigue and the relationship to pain, disease activity and quality of life in juvenile idiopathic arthritis and juvenile dermatomyositis.","volume":"50","author":"Butbul Aviel","year":"2011","journal-title":"Rheumatology."},{"key":"B24","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1002\/acr.21801","article-title":"Disease activity and fatigue in juvenile idiopathic arthritis.","volume":"65","author":"Ringold","year":"2013","journal-title":"Arthritis Care Res."},{"key":"B25","doi-asserted-by":"publisher","first-page":"80","DOI":"10.1186\/s12969-019-0370-1","article-title":"Health-related quality of life during early aggressive treatment in patients with polyarticular juvenile idiopathic arthritis: results from randomized controlled trial.","volume":"17","author":"Tarkiainen","year":"2019","journal-title":"Pediatr Rheumatol Online J."},{"key":"B26","doi-asserted-by":"publisher","first-page":"339","DOI":"10.1002\/msc.1239","article-title":"The correlation between the Childhood Health Assessment Questionnaire and disease activity in juvenile idiopathic arthritis.","volume":"16","author":"Sontichai","year":"2018","journal-title":"Musculoskelet Care."},{"key":"B27","doi-asserted-by":"publisher","first-page":"5954897","DOI":"10.1155\/2018\/5954897","article-title":"Influence of Biological Therapeutics, Cytokines, and Disease Activity on Depression in Rheumatoid Arthritis.","volume":"2018","author":"Figueiredo-Braga","year":"2018","journal-title":"J Immunol Res."},{"key":"B28","doi-asserted-by":"publisher","first-page":"34","DOI":"10.1186\/s41927-019-0080-9","article-title":"Health-related quality of life in patients with rheumatoid arthritis.","volume":"3","author":"Katchamart","year":"2019","journal-title":"BMC Rheumatol."},{"key":"B29","doi-asserted-by":"publisher","first-page":"1545","DOI":"10.17219\/acem\/104548","article-title":"Quality of life and clinical outcomes in Polish patients with high activity rheumatoid arthritis treated with leflunomide (Arava\u00ae) in Therapeutic Program: a retrospective analysis of data from the PLUS study.","volume":"28","author":"T\u0142ustochowicz","year":"2019","journal-title":"Adv Clin Exp Med."},{"key":"B30","doi-asserted-by":"publisher","first-page":"24009","DOI":"10.1002\/acr.24009","article-title":"Low Disease Activity State\/Remission Predicts a Better Health-Related Quality of Life in Systemic Lupus Erythematosus Patients.","volume":"72","author":"Ugarte-Gil","year":"2019","journal-title":"Arthritis Care Res."},{"key":"B31","doi-asserted-by":"publisher","first-page":"3032","DOI":"10.1093\/rheumatology\/keaa091","article-title":"Joint trajectories of disease activity, and physical and mental health-related quality of life in an inception lupus cohort.","volume":"59","author":"Fung","year":"2020","journal-title":"Rheumatology."},{"key":"B32","doi-asserted-by":"publisher","first-page":"1628","DOI":"10.1177\/0961203319886065","article-title":"Changes in quality of life in relation to disease activity in systemic lupus erythematosus: post-hoc analysis of the BLISS-52 Trial.","volume":"28","author":"Jolly","year":"2019","journal-title":"Lupus."},{"key":"B33","first-page":"535","article-title":"Clinical features of patients with systemic lupus erythematosus according to health-related quality of life, entity of pain, fatigue and depression: a cluster analysis.","volume":"37","author":"Margiotta","year":"2019","journal-title":"Clin Exp Rheumatol."},{"key":"B34","doi-asserted-by":"publisher","first-page":"8","DOI":"10.1186\/s13075-020-2095-4","article-title":"Comparison of performance of specific (SLEQOL) and generic (SF36) health-related quality of life questionnaires and their associations with disease status of systemic lupus erythematosus: a longitudinal study.","volume":"22","author":"Louthrenoo","year":"2020","journal-title":"Arthritis Res Ther."},{"key":"B35","doi-asserted-by":"publisher","first-page":"1398","DOI":"10.1093\/rheumatology\/kez451","article-title":"Disease-specific quality of life following a flare in systemic lupus erythematosus: an item response theory analysis of the French EQUAL cohort.","volume":"59","author":"Corneloup","year":"2019","journal-title":"Rheumatology."},{"key":"B36","doi-asserted-by":"publisher","first-page":"613","DOI":"10.1080\/09273948.2019.1616770","article-title":"Longitudinal Study Investigating the Relationship between Disease Activity and Psychological Status of Patients with Beh\u00e7et\u2019s Disease.","volume":"28","author":"Zulfiqar","year":"2019","journal-title":"Ocul Immunol Inflamm."},{"key":"B37","doi-asserted-by":"publisher","first-page":"4","DOI":"10.1186\/s12955-019-1260-4","article-title":"Relationship between disease activity status or clinical response and patient-reported outcomes in patients with non-radiographic axial spondyloarthritis: 104-week results from the randomized controlled EMBARK study.","volume":"18","author":"Dougados","year":"2020","journal-title":"Health Qual Life Outcomes."},{"key":"B38","doi-asserted-by":"publisher","first-page":"757","DOI":"10.1007\/s00296-020-04519-2","article-title":"The evaluation of anxiety, depression and quality of life scores of children and adolescents with familial Mediterranean fever.","volume":"40","author":"S\u00f6nmez","year":"2020","journal-title":"Rheumatol Int."},{"key":"B39","first-page":"355","article-title":"Quality of life of patients with familial Mediterranean fever.","volume":"15","author":"Buskila","year":"1997","journal-title":"Clin Exp Rheumatol."},{"key":"B40","doi-asserted-by":"publisher","first-page":"347","DOI":"10.1097\/pep.0000000000000052","article-title":"Functional capacity, strength, and quality of life in children and youth with familial Mediterranean fever.","volume":"26","author":"Alayli","year":"2014","journal-title":"Pediatr Phys Ther."},{"key":"B41","first-page":"958","article-title":"Assesment life quality of familial Mediterranean fever patients by short form-36 and its relationship with disease parameters.","volume":"17","author":"Sahin","year":"2013","journal-title":"Eur Rev Med Pharmacol Sci."},{"key":"B42","doi-asserted-by":"publisher","first-page":"541","DOI":"10.1515\/ijamh-2013-0332","article-title":"Quality of life measures and psychiatric symptoms in adolescents with systemic lupus erythematosus and familial Mediterranean fever.","volume":"26","author":"D\u00fcz\u00e7eker","year":"2014","journal-title":"Int J Adolesc Med Health."},{"key":"B43","doi-asserted-by":"publisher","first-page":"375","DOI":"10.1007\/s10067-009-1330-9","article-title":"Depression and anxiety in children and adolescents with familial Mediterranean fever.","volume":"29","author":"Makay","year":"2010","journal-title":"Clin Rheumatol."},{"key":"B44","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1007\/s00296-020-04519-2","article-title":"The evaluation of anxiety, depression and quality of life scores of children and adolescents with familial Mediterranean fever.","volume":"40","author":"Sonmez","year":"2020","journal-title":"Rheumatol Int."},{"key":"B45","doi-asserted-by":"publisher","first-page":"1849","DOI":"10.1111\/1756-185X.13104","article-title":"Incidence of sleep disturbances in patients with familial Mediterranean fever and the relation of sleep quality with disease activity.","volume":"21","author":"Kucuksahin","year":"2018","journal-title":"Int J Rheumat Dis."},{"key":"B46","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1007\/s00296-017-3882-3","article-title":"Fatigue in familial Mediterranean fever and its relations with other clinical parameters.","volume":"38","author":"Duruoz","year":"2018","journal-title":"Rheumatol Int."},{"key":"B47","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1016\/j.ejr.2017.07.004","article-title":"Quality of life in Turkish patients with Familial Mediterranean Fever: association with fatigue, psychological status, disease severity and other clinical parameters.","volume":"40","author":"Guler","year":"2018","journal-title":"Egypt Rheumatol."}],"container-title":["Frontiers in Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.822473\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,27]],"date-time":"2022-04-27T18:26:07Z","timestamp":1651083967000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fped.2022.822473\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,4,27]]},"references-count":47,"alternative-id":["10.3389\/fped.2022.822473"],"URL":"https:\/\/doi.org\/10.3389\/fped.2022.822473","relation":{},"ISSN":["2296-2360"],"issn-type":[{"value":"2296-2360","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,4,27]]},"article-number":"822473"}}