title: Performance of Different Diagnostic Criteria for Familial Mediterranean Fever in Children with Periodic Fevers: Results from a Multicenter International Registry.
abstract: OBJECTIVE
  Our aims were to validate the pediatric diagnostic criteria in a large international registry and to compare them with the performance of previous criteria for the diagnosis of familial Mediterranean fever (FMF).
  
  
  METHODS
  Pediatric patients with FMF from the Eurofever registry were used for the validation of the existing criteria. The other periodic fevers served as controls: mevalonate kinase deficiency (MKD), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), cryopyrin-associated periodic syndrome (CAPS), aphthous stomatitis, pharyngitis, adenitis syndrome (PFAPA), and undefined periodic fever from the same registry. The performances of Tel Hashomer, Livneh, and the Yalcinkaya-Ozen criteria were assessed.
  
  
  RESULTS
  The FMF group included 339 patients. The control group consisted of 377 patients (53 TRAPS, 45 MKD, 32 CAPS, 160 PFAPA, 87 undefined periodic fevers). Patients with FMF were correctly diagnosed using the Yalcinkaya-Ozen criteria with a sensitivity rate of 87.4% and a specificity rate of 40.7%. On the other hand, Tel Hashomer and Livneh criteria displayed a sensitivity of 45.0 and 77.3%, respectively. Both of the latter criteria displayed a better specificity than the Yalcinkaya-Ozen criteria: 97.2 and 41.1% for the Tel Hashomer and Livneh criteria, respectively. The overall accuracy for the Yalcinkaya-Ozen criteria was 65 and 69.6% (using 2 and 3 criteria), respectively. Ethnicity and residence had no effect on the performance of the Yalcinkaya-Ozen criteria.
  
  
  CONCLUSIONS
  The Yalcinkaya-Ozen criteria yielded a better sensitivity than the other criteria in this international cohort of patients and thus can be used as a tool for FMF diagnosis in pediatric patients from either the European or eastern Mediterranean region. However, the specificity was lower than the previously suggested adult criteria.
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Erkan
authors.initials: E
authors.lastname: Demirkaya
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Celal
authors.initials: C
authors.lastname: Saglam
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Turker
authors.initials: T
authors.lastname: Turker
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Isabelle
authors.initials: I
authors.lastname: Koné-Paut
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Pat
authors.initials: P
authors.lastname: Woo
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Matteo
authors.initials: M
authors.lastname: Doglio
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Gayane
authors.initials: G
authors.lastname: Amaryan
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Joost
authors.initials: J
authors.lastname: Frenkel
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Yosef
authors.initials: Y
authors.lastname: Uziel
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Antonella
authors.initials: A
authors.lastname: Insalaco
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Michael
authors.initials: M
authors.lastname: Hofer
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Sorina
authors.initials: S
authors.lastname: Boiu
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Ali
authors.initials: A
authors.lastname: Duzova
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Consuelo
authors.initials: C
authors.lastname: Modesto
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Annette
authors.initials: A
authors.lastname: Bryant
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Efimia
authors.initials: E
authors.lastname: Papadopoulou-Alataki
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Severine
authors.initials: S
authors.lastname: Guillaume-Czitrom
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Jasmine
authors.initials: J
authors.lastname: Kuemmerle-Deschner
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Bénédicte
authors.initials: B
authors.lastname: Neven
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Helen
authors.initials: H
authors.lastname: Lachmann
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Alberto
authors.initials: A
authors.lastname: Martini
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Nicolino
authors.initials: N
authors.lastname: Ruperto
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Marco
authors.initials: M
authors.lastname: Gattorno
authors.affiliation: From the Familial Mediterranean Fever Arthritis Vasculitis and Orphan Disease Research Center (FAVOR), Gulhane Military Medical Faculty; Epidemiology, Gulhane Military Medical Faculty, Ankara, Turkey; Centre de référence national des maladies auto-inflammatoires, CEREMAI, Rhumatologie pédiatrique, CHU Le Kremlin Bicêtre (APHP, University of Paris SUD), Paris, France; Center of Paediatric and Adolescent Rheumatology, UCL, London, UK; Istituto Giannina Gaslini, UO Pediatria II, Reumatologia, Genoa, Italy; Arabkir Medical Centre, Institute of Child and Adolescents Health, Yerevan, Armenia; Department of Paediatrics, University Medical Center Utrecht, Utrecht, Netherlands; Pediatrics, Meir Medical Centre, Kfar Saba, Israel; Division of Rheumatology, Department of Pediatric Medicine, Bambino Gesù Children's Hospital, IRCCS, Rome; Rheumatology Unit, Policlinico le Scotte, University of Siena, Siena, Italy; Pediatrie, Centre Multisite Romand de Rhumatologie Pediatrique/Centre Hospitalier, Universitaire Vaudois (CHUV), Lausanne, Switzerland; Pediatric Rheumatology, Université Paris-Descartes, Paris, France; Pediatric Rheumatology, Hacettepe University, School of Medicine, Ankara, Turkey; Reumatologia, Hospital Valle de Hebron, Barcelona, Spain; Pediatrics, Università Cattolica Sacro Cuore, Rome, Italy; Fourth Department of Pediatrics, Aristotle University of Thessaloniki Papageorgiou Hospital, Thessaloniki, Greece; Universitatsklinik fur Kinderheilkunde und Jugendmedizin, Tübingen, Germany; Université Paris-Descartes, Hôpital Necker-Enfants Malades, Centre de référence national pour les Arthrites Juveniles, Unité d'Immunologie, Hématologie et Rhumatologie Pédiatrique, Université Descartes, Sorbonne Paris Cité, Institut IMAGINE, Paris, France; National Amyloidosis Centre, University College London Medical School, Royal Free Campus, London, UK.E. Demirkaya, MD, MSc, FAVOR, Gulhane Military Medical Faculty; C. Saglam, MD, FAVOR, Gulhane Military Medical Facult
authors.forename: Seza
authors.initials: S
authors.lastname: Ozen
mesh_terms: D016009:Chi-Square Distribution; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003937:Diagnosis, Differential; D003955:Diagnostic Tests, Routine / Q000379:methods*; D005060:Europe; D010505:Familial Mediterranean Fever / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology; D005260:Female; D005334:Fever / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology; D056660:Hereditary Autoinflammatory Diseases / Q000145:classification / Q000175:diagnosis* / Q000453:epidemiology; D006801:Humans; D038622:Internationality; D008297:Male; D012042:Registries; D012189:Retrospective Studies; D012720:Severity of Illness Index; D018709:Statistics, Nonparametric
keywords: AUTOINFLAMMATORY DISEASES; CHILDREN; FAMILIAL MEDITERRANEAN FEVER; LIVNEH CRITERIA; TEL HASHOMER CRITERIA; YALCINKAYA-OZEN CRITERIA