Evidence map›Paper›PMID 36881285›Full record

Trial reportInternal and emergency medicine2023

Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry.

Carla Gaggiano, Anna Maselli, Petros P Sfikakis, Katerina Laskari, Gaafar Ragab, Mohamed Tharwat Hegazy, Ahmed Hatem Laymouna, Giuseppe Lopalco, Ibrahim A Almaghlouth, Kazi Nur Asfina and 47 more

Registry-linked trialOpen access · hybridAbstract readClinical Trial
PubMed Full text at PMC Publisher
In one paragraph

Trial report in Internal and emergency medicine, 2023. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT05200715 (Development of an International Multicenter Registry of Patients With Monogenic and Polygenic Autoinflammatory Diseases Aimed at Clinical and Therapeutical Data Collection and Analysis), which is not on this map. Cited by 3 papers.

0numbers the graph read from it
0cells of the map it votes in
3citing papers in PubMed
2.8field-weighted citation impact, top 10% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

NCT05200715 recruitingnot on this map

Development of an International Multicenter Registry of Patients With Monogenic and Polygenic Autoinflammatory Diseases Aimed at Clinical and Therapeutical Data Collection and Analysis

Typeobservational_patient_registrySponsorUniversity of SienaRan2020 to 2030Enrolled3,500ConditionsHereditary Autoinflammatory Diseases, Schnitzler Syndrome, Behcet Syndrome, PFAPA SyndromeArmsNo intervention is foreseen by the protocol.
3 · Its place in the literature

Who cites it

3 citing papers in PubMed, 10 citations in OpenAlex.

  1. Article
  2. Pivotal role of the synovioentheseal complex in the imaging of arthritis and rheumatic diseasesDiagnostic and interventional radiology (Ankara, Turkey) · 2024
    Review
  3. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

57 authors at 20 institutions in 11 countries.

Carla GaggianoDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Anna MaselliClinical Paediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy.
Petros P SfikakisJoint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Katerina LaskariJoint Academic Rheumatology Program, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
Gaafar RagabInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Mohamed Tharwat HegazyInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Ahmed Hatem LaymounaInternal Medicine Department, Rheumatology and Clinical Immunology Unit, Faculty of Medicine, Cairo University, Giza, Egypt.
Giuseppe LopalcoRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
Ibrahim A AlmaghlouthRheumatology Unit, Department of Medicine, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Kazi Nur AsfinaCollege of Medicine Research Center, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Ohoud AlahmedPediatric Rheumatology, Department of Pediatrics, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Henrique Ayres Giardini MayrinkRheumatology Division, Faculdade de Medicina, Hospital das Clinicas (HCFMUSP), Universidade de Sao Paulo, São Paulo, Brazil.
Isabele Parente de Brito AntonelliRheumatology Division, Faculdade de Medicina, Hospital das Clinicas (HCFMUSP), Universidade de Sao Paulo, São Paulo, Brazil.
Marco CattaliniPediatric Clinic, University of Brescia and Spedali Civili Di Brescia [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy.
Matteo PigaRheumatology Unit, Department of Medical Sciences, University and AOU of Cagliari, Cagliari, Italy.
Jurgen SotaDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Stefano GentileschiRheumatology Unit, Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Maria Cristina MaggioUniversity Department of Health Promotion, Mother and Child Care, Internal Medicine and Medical Specialties (PROMISE) "G. D'Alessandro", University of Palermo, Palermo, Italy.
Daniela Opris-BelinskiRheumatology and Internal Medicine Department, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
Gülen HatemiDepartment of Internal Medicine, Division of Rheumatology, Cerrahpasa Medical School, Istanbul University-Cerrahpasa, Istanbul, Turkey.
Antonella InsalacoDivision of Rheumatology, Ospedale Pediatrico Bambino Gesù, IRCCS [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Rome, Italy.
Alma Nunzia OlivieriDepartment of Woman, Child and of General and Specialized Surgery, University of Campania "Luigi Vanvitelli", Naples, Italy.
Abdurrahman TufanDivision of Rheumatology, Department of Internal Medicine, Faculty of Medicine, Gazi University, Ankara, Turkey.
Hazan KaradenizDepartment of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey.
Riza Can KardaşDepartment of Internal Medicine, Division of Rheumatology, Gazi University Hospital, Ankara, Turkey.
Francesco La TorreDepartment of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
Fabio CardinaleDepartment of Pediatrics, Pediatric Rheumatology Center, Giovanni XXIII Pediatric Hospital, University of Bari, Bari, Italy.
Achille MarinoUnit of Pediatric Rheumatology, Azienda Socio-Sanitaria Territoriale (ASST) Gaetano Pini-Centro Specialistico Ortopedico Traumatologico (CTO), Milan, Italy.
Silvana GuerrieroDepartment of Ophthalmology and Otolaryngology, University of Bari, Bari, Italy.
Piero RuscittiRheumatology Unit, Department of Biotechnological and Applied Clinical Sciences, University of L'Aquila, L'Aquila, Italy.
Maria TarsiaClinical Paediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy.
Antonio VitaleDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Valeria CaggianoDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Salvatore TelescaDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Florenzo IannoneRheumatology Unit, Department of Emergency and Organ Transplantation, University of Bari, Bari, Italy.
Veronica ParrettiDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy.
Micol FrassiRheumatology and Clinical Immunology, Department of Clinical and Experimental Sciences, University of Brescia and Spedali Civili [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Brescia, Italy.
Emma AragonaDivision of Gastroenterology, Ospedali Riuniti Villa Sofia-Vincenzo Cervello, Palermo, Italy.
Francesco CicciaDepartment of Precision Medicine, Università Degli Studi Della Campania Luigi Vanvitelli, Naples, Italy.
Ewa Wiesik-SzewczykDepartment of Internal Medicine, Pulmonology, Allergy and Clinical Immunology, Central Clinical Hospital of the Ministry of National Defence, Military Institute of Medicine, Warsaw, Poland.
Ruxandra IonescuInternal Medicine and Rheumatology Department-St. Maria Hospital, Carol Davila University of Medicine and Pharmacy, Bucharest, Romania.
Ali ŞahinDivision of Rheumatology, Department of Internal Medicine, Sivas Cumhuriyet University Medical Faculty, Sivas, Turkey.
Nurullah AkkoçDivision of Rheumatology, Department of Internal Medicine, School of Medicine, Manisa Celal Bayar University, Manisa, Turkey.
Andrea Hinojosa-AzaolaDepartment of Immunology and Rheumatology, Instituto Nacional de Ciencias Médicas Y Nutrición Salvador Zubirán, Mexico City, Mexico.
Samar TharwatRheumatology and Immunology Unit, Internal Medicine Department, Mansoura University, Mansoura, Egypt.
José Hernández-RodríguezDepartment of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain.
Gerard EspinosaDepartment of Autoimmune Diseases, Institut d'Investigacions Biomèdiques August Pi I Sunyer (IDIBAPS), Hospital Clínic of Barcelona [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], University of Barcelona, Barcelona, Spain.
Giovanni ContiPediatric Nephrology and Rheumatology Unit, Azienda Ospedaliero Universitaria (AOU) G Martino, Messina, Italy.
Emanuela Del GiudicePediatric and Neonatology Unit, Department of Maternal Infantile and Urological Sciences, Sapienza University of Rome, Polo Pontino, Latina, Italy.
Marcello GovoniDepartment of Medical Sciences, Rheumatology Unit, Azienda Ospedaliero-Universitaria S. Anna-Ferrara, University of Ferrara, Ferrara, Italy.
Giacomo EmmiDepartment of Experimental and Clinical Medicine, University of Florence, Florence, Italy.
Claudia FabianiOphthalmology Unit, Department of Medicine, Surgery and Neurosciences, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Alberto BalistreriBioengineering and Biomedical Data Science Lab, Department of Medical Biotechnologies, University of Siena, Siena, Italy.
Bruno FredianiRheumatology Unit, Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Siena, Italy.
Donato RiganteDepartment of Life Sciences and Global Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.
Luca CantariniDepartment of Medical Sciences, Surgery and Neurosciences, Rheumatology Unit, University of Siena and Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center], Policlinico "Le Scotte", Viale Bracci 16, 53100, Siena, Italy. cantariniluca@hotmail.com.ORCID 0000-0002-7352-1275
AIDA Network
University of Siena · ITCairo University · EGGazi University · TRKing Saud University · SAUniversity of Bari Aldo Moro · ITCarol Davila University of Medicine and Pharmacy · ROConsorci Institut D'Investigacions Biomediques August Pi I Sunyer · ESHospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo · BRNational and Kapodistrian University of Athens · GROspedale Pediatrico Giovanni XXIII · ITUniversity of Brescia · ITUniversity of Campania "Luigi Vanvitelli" · ITAzienda Ospedaliera Universitaria Policlinico "G. Martino" · ITAzienda Ospedaliera Universitaria Senese · ITBambino Gesù Children's Hospital · ITCentral Clinical Hospital · PLInstituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán · MXIstanbul University-Cerrahpaşa · TRIstituto Ortopedico Gaetano Pini · ITManisa Celal Bayar University · TR

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet's syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet's Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0-4). Colchicine was inefficacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p = 0.46) or the concomitant therapy (p = 0.30 for cDMARDs, p = 1.00 for glucocorticoids); cDMARDs and bDMARDs were inefficacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefficacy (p = 0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively affects response to biologic therapies. ClinicalTrials.gov Identifier: NCT05200715 (registered on December 18, 2021).

Indexed as

ArthritisBehcet SyndromeChildHumansMyalgiaRegistriesUlcerArthritisBehçet’s syndromeInternational registryPediatric rheumatologyRare diseases

Identifiers

PMID36881285
PMCPMC10082129
OpenAlexW4323347834

What OpenQuestion holds

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LicenceCC BY
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Registered trials

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.