{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,11]],"date-time":"2026-07-11T17:58:37Z","timestamp":1783792717877,"version":"3.55.0"},"reference-count":33,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2019,11,18]],"date-time":"2019-11-18T00:00:00Z","timestamp":1574035200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2019,11,18]],"date-time":"2019-11-18T00:00:00Z","timestamp":1574035200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Rheumatol Int"],"published-print":{"date-parts":[[2020,1]]},"DOI":"10.1007\/s00296-019-04478-3","type":"journal-article","created":{"date-parts":[[2019,11,18]],"date-time":"2019-11-18T06:01:56Z","timestamp":1574056916000},"page":"49-56","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Pattern and diagnostic evaluation of systemic autoinflammatory diseases other than familial Mediterranean fever among Arab children: a multicenter study from the Pediatric Rheumatology Arab Group (PRAG)"],"prefix":"10.1007","volume":"40","author":[{"name":"for Pediatric Arab Rheumatology Group (PRAG)","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0142-6698","authenticated-orcid":false,"given":"Sulaiman M.","family":"Al-Mayouf","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8091-3226","authenticated-orcid":false,"given":"Abdulaziz","family":"Almutairi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8303-4991","authenticated-orcid":false,"given":"Safiya","family":"Albrawi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5964-9088","authenticated-orcid":false,"given":"Basil M.","family":"Fathalla","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4972-5809","authenticated-orcid":false,"given":"Raed","family":"Alzyoud","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0429-5099","authenticated-orcid":false,"given":"Abdullatif","family":"AlEnazi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3146-839X","authenticated-orcid":false,"given":"Mohammed","family":"Abu-shukair","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1800-9786","authenticated-orcid":false,"given":"Adel","family":"Alwahadneh","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0761-8378","authenticated-orcid":false,"given":"Abdullah","family":"Alsonbul","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4960-2312","authenticated-orcid":false,"given":"Mabruka","family":"Zlenti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9215-292X","authenticated-orcid":false,"given":"Ebtisam","family":"Khawaja","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3149-1266","authenticated-orcid":false,"given":"Awatif","family":"Abushhaiwia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4432-108X","authenticated-orcid":false,"given":"Khulood","family":"Khawaja","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6109-3656","authenticated-orcid":false,"given":"Zakiya","family":"AlMosawi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3738-3629","authenticated-orcid":false,"given":"Wafa","family":"Madan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2199-7159","authenticated-orcid":false,"given":"Muna","family":"Almuatiri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5249-9144","authenticated-orcid":false,"given":"Nora","family":"Almuatiri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,11,18]]},"reference":[{"key":"4478_CR1","doi-asserted-by":"publisher","first-page":"261","DOI":"10.2174\/09298673113206660303","volume":"21","author":"F Ciccarelli","year":"2014","unstructured":"Ciccarelli F, De Martinis F, Ginaldi L (2014) An update on autoinflammatory diseases. Curr Med Chem 21:261\u2013269","journal-title":"Curr Med Chem"},{"key":"4478_CR2","doi-asserted-by":"publisher","first-page":"701","DOI":"10.1016\/j.rdc.2013.08.001","volume":"39","author":"G Sanchez","year":"2013","unstructured":"Sanchez G, de Jesus A, Goldbach-Mansky R (2013) Monogenic autoinflammatory diseases: disorders of amplified danger sensing and cytokine dysregulation. Rheum Dis Clin N Am 39:701\u2013734","journal-title":"Rheum Dis Clin N Am"},{"key":"4478_CR3","doi-asserted-by":"publisher","first-page":"799","DOI":"10.1136\/annrheumdis-2014-206580","volume":"74","author":"S Federici","year":"2015","unstructured":"Federici S, Sormani M, Ozen S, Lachmann H, Amaryan G, Woo P et al (2015) Evidence-based provisional clinical classification criteria for autoinflammatory periodic fevers. Ann Rheum Dis 74:799\u2013805","journal-title":"Ann Rheum Dis"},{"key":"4478_CR4","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1136\/jclinpath-2016-203810","volume":"70","author":"S Pathak","year":"2017","unstructured":"Pathak S, McDermott M, Savic S (2017) Autoinflammatory diseases: update on classification diagnosis and management. J Clin Pathol 70:1\u20138","journal-title":"J Clin Pathol"},{"key":"4478_CR5","doi-asserted-by":"publisher","first-page":"349","DOI":"10.1136\/jmedgenet-2013-101577","volume":"50","author":"I Touitou","year":"2013","unstructured":"Touitou I (2013) Inheritance of autoinflammatory diseases: shifting paradigms and nomenclature. J Med Genet 50:349\u2013359","journal-title":"J Med Genet"},{"key":"4478_CR6","doi-asserted-by":"publisher","first-page":"403","DOI":"10.1007\/s00281-015-0498-0","volume":"37","author":"M Omberllo","year":"2015","unstructured":"Omberllo M (2015) Advances in the genetically complex autoinflammatory diseases. Semin Immunopathol 37:403\u2013406","journal-title":"Semin Immunopathol"},{"key":"4478_CR7","doi-asserted-by":"publisher","first-page":"1177","DOI":"10.1136\/annrheumdis-2011-200549","volume":"71","author":"N Toplak","year":"2012","unstructured":"Toplak N, Frenkel J, Ozen S, Lachmann H, Woo P, Kone-Paut I et al (2012) An international registry on autoinflammatory diseases: the Eurofever experience. Ann Rheum Dis 71:1177\u20131182","journal-title":"Ann Rheum Dis"},{"key":"4478_CR8","doi-asserted-by":"publisher","first-page":"21","DOI":"10.5152\/eurjrheum.2014.006","volume":"1","author":"I Sari","year":"2014","unstructured":"Sari I, Birlik M, Kasifo\u011flu T (2014) Familial mediterranean fever: an updated review. Eur J Rheumatol 1:21\u201333","journal-title":"Eur J Rheumatol"},{"key":"4478_CR9","doi-asserted-by":"publisher","DOI":"10.1155\/2019\/2578760","author":"A Mansour","year":"2019","unstructured":"Mansour A, El-Shayeb A, El Habachi N, Khodair M, Elwazzan D, Abdeen N et al (2019) Molecular patterns of MEFV gene mutations in egyptian patients with familial mediterranean fever: a retrospective cohort study. Int J Inflam. https:\/\/doi.org\/10.1155\/2019\/2578760(eCollection 2019)","journal-title":"Int J Inflam"},{"key":"4478_CR10","doi-asserted-by":"publisher","first-page":"981","DOI":"10.1007\/s00296-010-1732-7","volume":"32","author":"L Belmahi","year":"2012","unstructured":"Belmahi L, Cherkaoui I, Hama I, Sefiani A (2012) MEFV mutations in Moroccan patients suffering from familial mediterranean fever. Rheumatol Int 32:981\u2013984","journal-title":"Rheumatol Int"},{"key":"4478_CR11","doi-asserted-by":"publisher","first-page":"43","DOI":"10.5144\/0256-4947.2012.43","volume":"32","author":"A Alenazi","year":"2012","unstructured":"Alenazi A, Al Sonbul A, Al Jumaah S, Al Mehaidib A, Al-Mayouf SM (2012) A retrospective review of autoinflammatory diseases in Saudi children at a rheumatology clinic. Ann Saudi Med 32:43\u201348","journal-title":"Ann Saudi Med"},{"key":"4478_CR12","doi-asserted-by":"publisher","first-page":"1599","DOI":"10.1136\/annrheumdis-2018-213725","volume":"77","author":"N Ter Haar","year":"2018","unstructured":"Ter Haar N, van Delft A, Annink K, van Stel H, Al-Mayouf SM, Amaryan G et al (2018) In silico validation of the autoinflammatory disease damage index. Ann Rheum Dis 77:1599\u20131605","journal-title":"Ann Rheum Dis"},{"key":"4478_CR13","doi-asserted-by":"publisher","first-page":"288","DOI":"10.1002\/art.38877","volume":"67","author":"S Wakil","year":"2015","unstructured":"Wakil S, Monies D, Abodelhoda M, Al-Tassan N, Al-Dusery H, Naim E et al (2015) Association of a mutation in LACC1 with a monogenic form of systemic juvenile idiopathic arthritis. Arthritis Rheumatol 67:288\u2013295","journal-title":"Arthritis Rheumatol"},{"key":"4478_CR14","doi-asserted-by":"publisher","first-page":"1831","DOI":"10.1136\/gutjnl-2014-307859","volume":"63","author":"N Patel","year":"2014","unstructured":"Patel N, El-Mouzan M, Al-Mayouf SM, Adly N, Mohamed J, AlMofarreh M et al (2014) Study of Mendelian forms of Crohn\u2019s disease in Saudi Arabia reveals novel risk loci and alleles. Gut 63:1831\u20131832","journal-title":"Gut"},{"key":"4478_CR15","doi-asserted-by":"publisher","first-page":"249","DOI":"10.1007\/s10875-015-0140-x","volume":"35","author":"T Moussa","year":"2015","unstructured":"Moussa T, Aladbe B, Taha R, Remmers E, El-Shanti H, Fathalla B (2015) Overlap of familial Mediterranean fever and hyper-IgD syndrome in Arabic kindred. J Clin Immunol 35:249\u2013253","journal-title":"J Clin Immunol"},{"key":"4478_CR16","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1111\/1756-185X.13228","volume":"21","author":"SM Al-Mayouf","year":"2018","unstructured":"Al-Mayouf SM, AlSaleem A, AlMutairi N, AlSonbul A, Alzaid T, Alazami A, Al-Mousa H (2018) Monogenic interferonopathies: phenotypic and genotypic findings of CANDLE syndrome and its overlap with C1q deficient SLE. Int J Rheum Dis 21:208\u2013213","journal-title":"Int J Rheum Dis"},{"key":"4478_CR17","first-page":"956","volume":"32","author":"B Fathalla","year":"2014","unstructured":"Fathalla B, Al-Wahadenh A, Al-Mutawa M, Kambouris M, El-Shanti H (2014) A novel de novo PSTPIP1 mutation in a boy with pyogenic arthritis, pyoderma gangrenosum, acne (PAPA) syndrome. Clin Exp Rheumatol 32:956\u2013958","journal-title":"Clin Exp Rheumatol"},{"key":"4478_CR18","doi-asserted-by":"publisher","first-page":"612","DOI":"10.1136\/annrheumdis-2017-212401","volume":"77","author":"A Giannelou","year":"2018","unstructured":"Giannelou A, Wang H, Zhou Q, Park Y, Abu-Asab M, Ylaya K et al (2018) Aberrant tRNA processing causes an autoinflammatory syndrome responsive to TNF inhibitors. Ann Rheum Dis 77:612\u2013619","journal-title":"Ann Rheum Dis"},{"key":"4478_CR19","doi-asserted-by":"publisher","first-page":"1558","DOI":"10.1136\/annrheumdis-2017-212515","volume":"77","author":"E Ben-Chetrit","year":"2018","unstructured":"Ben-Chetrit E, Gattorno M, Gul A, Kastner D, Lachmann H, Touitou I et al (2018) Consensus proposal for taxonomy and definition of the autoinflammatory diseases (AIDs): a Delphi study. Ann Rheum Dis 77:1558\u20131565","journal-title":"Ann Rheum Dis"},{"key":"4478_CR20","doi-asserted-by":"publisher","first-page":"154","DOI":"10.3899\/jrheum.141249","volume":"43","author":"E Demirkaya","year":"2016","unstructured":"Demirkaya E, Saglam C, Turker T, Kone-Paut I, Woo P, Doglio M et al (2016) Performance of different diagnostic criteria for familial Mediterranean fever in children with periodic fevers: results from a multicenter international registry. J Rheumatol 43:154\u2013160","journal-title":"J Rheumatol"},{"key":"4478_CR21","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2019-215048","author":"M Gattorno","year":"2019","unstructured":"Gattorno M, Hofer M, Federici S, Vanoni F, Bovis F, Aksentijevich I et al (2019) Classification criteria for autoinflammatory recurrent fevers. Ann Rheum Dis. https:\/\/doi.org\/10.1136\/annrheumdis-2019-215048","journal-title":"Ann Rheum Dis"},{"key":"4478_CR22","doi-asserted-by":"publisher","first-page":"187","DOI":"10.1097\/BOR.0000000000000362","volume":"29","author":"I Aksentijevich","year":"2017","unstructured":"Aksentijevich I, McDermott M (2017) Lessons from characterization and treatment of the inflammatory syndromes. Curr Opin Rheumatol 29:187\u2013194","journal-title":"Curr Opin Rheumatol"},{"key":"4478_CR23","doi-asserted-by":"publisher","first-page":"2795","DOI":"10.1002\/art.39763","volume":"68","author":"N Ter Haar","year":"2016","unstructured":"Ter Haar N, Jeyaratnam J, Lachmann H, Simon A, Brogan P, Doglio M et al (2016) The phenotype and genotype of mevalonate kinase deficiency: a series of 114 cases from the Eurofever registry. Arthritis Rheumatol 68:2795\u20132805","journal-title":"Arthritis Rheumatol"},{"key":"4478_CR24","doi-asserted-by":"publisher","DOI":"10.1186\/s13023-017-0720-3","author":"R Papa","year":"2017","unstructured":"Papa R, Doglio M, Lachmann H, Ozen S, Frenkel J, Simon A et al (2017) A web-based collection of genotype-phenotype associations in hereditary recurrent fevers from the Eurofever registry. Orphanet J Rare Dis. https:\/\/doi.org\/10.1186\/s13023-017-0720-3","journal-title":"Orphanet J Rare Dis"},{"key":"4478_CR25","doi-asserted-by":"publisher","first-page":"942","DOI":"10.1136\/annrheumdis-2016-209686","volume":"76","author":"JB Kuemmerle-Deschner","year":"2017","unstructured":"Kuemmerle-Deschner JB, Ozen S, Tyrrell P (2017) Diagnostic criteria for cryopyrin-associated periodic syndrome (CAPS). Ann Rheum Dis 76:942\u2013947","journal-title":"Ann Rheum Dis"},{"key":"4478_CR26","doi-asserted-by":"publisher","first-page":"454","DOI":"10.1007\/s12016-017-8613-8","volume":"54","author":"Y Jamilloux","year":"2018","unstructured":"Jamilloux Y, Belot A, Magnotti F, Benezech S, Grefaud-Valentin M, Bourdonnay E et al (2018) Geoepidemiology and immunology features of autoinflammatory diseases: a comprehensive review. Clin Rev Allergy Immunol 54:454\u2013479","journal-title":"Clin Rev Allergy Immunol"},{"key":"4478_CR27","doi-asserted-by":"publisher","DOI":"10.1016\/j.waojou.2019.100019","author":"M Chuamanochan","year":"2019","unstructured":"Chuamanochan M, Weller K, Feist E, Kallinich T, Maurer M, Kuemmerle-Deschner J et al (2019) State of care for patients with systemic autoinflammatory diseases\u2014results of a tertiary care survey. World Allergy Organ J. https:\/\/doi.org\/10.1016\/j.waojou.2019.100019","journal-title":"World Allergy Organ J"},{"key":"4478_CR28","doi-asserted-by":"publisher","first-page":"417","DOI":"10.5455\/medarh.2015.69.417-420","volume":"69","author":"L Habahbeh","year":"2015","unstructured":"Habahbeh L, Hiary M, Zaben S, Al-Momani A, Khasawneh R, Mallouh M et al (2015) Genetic profile of patients with familial mediterranean fever (FMF): single center experience at King Hussein Medical Center (KHMC). Med Arch 69:417\u2013420","journal-title":"Med Arch"},{"key":"4478_CR29","doi-asserted-by":"publisher","first-page":"149","DOI":"10.1016\/j.ejmg.2016.12.003","volume":"60","author":"D Ait-Idir","year":"2017","unstructured":"Ait-Idir D, Djerdjouri B, Bouldjennet F, Taha R, El-Shanti H, Sari-Hamidou R et al (2017) The M694I\/M694I genotype: a genetic risk factor of AA-amyloidosis in a group of Algerian patients with familial Mediterranean fever. Eur J Med Genet. 60:149\u2013153","journal-title":"Eur J Med Genet."},{"key":"4478_CR30","doi-asserted-by":"publisher","DOI":"10.1186\/1742-4755-6-17","author":"G Tadmouri","year":"2009","unstructured":"Tadmouri G, Nair P, Obeid T, Al Ali M, Al Khaja N, Hamamy H (2009) Consanguinity and reproductive health among Arabs. Reprod Health. https:\/\/doi.org\/10.1186\/1742-4755-6-17","journal-title":"Reprod Health"},{"key":"4478_CR31","doi-asserted-by":"publisher","first-page":"1405","DOI":"10.1136\/annrheumdis-2018-214472","volume":"78","author":"NM Ter Haar","year":"2019","unstructured":"Ter Haar NM, Eijkelboom C, Cantarini L, Papa R, Brogan PA, Kone-Paut I et al (2019) Clinical characteristics and genetic analyses of 187 patients with undefined autoinflammatory diseases. Ann Rheum Dis. 78:1405\u20131411","journal-title":"Ann Rheum Dis."},{"key":"4478_CR32","doi-asserted-by":"publisher","DOI":"10.1186\/1546-0096-8-29","author":"N Toplak","year":"2010","unstructured":"Toplak N, Dolezalova P, Constantin T, Sediva A, Pasic S, Cinzar P et al (2010) Periodic fever syndromes in Eastern and Central European countries: results of a pediatric multinational survey. Pediatr Rheumatol Online J. https:\/\/doi.org\/10.1186\/1546-0096-8-29","journal-title":"Pediatr Rheumatol Online J"},{"key":"4478_CR33","doi-asserted-by":"publisher","first-page":"578","DOI":"10.1002\/acr.23120","volume":"69","author":"S Ozen","year":"2017","unstructured":"Ozen S, Kuemmerle-Deschner J, Cimaz R, Livneth A, Quartier P, Kon-Paut I et al (2017) International retrospective chart review of treatment patterns in severe familial Mediterranean fever, tumor necrosis factor receptor-associated periodic syndrome, and mevalonate kinase deficiency\/hyperimmunoglobulinemia D syndrome. Arthritis Care Res (Hoboken) 69:578\u2013586","journal-title":"Arthritis Care Res (Hoboken)"}],"container-title":["Rheumatology International"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00296-019-04478-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00296-019-04478-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00296-019-04478-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,11,17]],"date-time":"2020-11-17T00:16:29Z","timestamp":1605572189000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00296-019-04478-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,11,18]]},"references-count":33,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2020,1]]}},"alternative-id":["4478"],"URL":"https:\/\/doi.org\/10.1007\/s00296-019-04478-3","relation":{},"ISSN":["0172-8172","1437-160X"],"issn-type":[{"value":"0172-8172","type":"print"},{"value":"1437-160X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,11,18]]},"assertion":[{"value":"8 August 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 November 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 November 2019","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with ethical standards"}},{"value":"All authors declare that they have no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. Taking in consideration the nature of this retrospective work, informed consent for participation in this study was not required. However, an informed consent for genetic testing as part of patient care was obtained from patient or parent.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}]}}