Evidence map›Paper›PMID 40748718›Full record

ArticleRheumatology (Oxford, England)2026

Biological therapies in paediatric Behçet's disease: results of an international collaborative study by the PRES Vasculitis Working Party.

Özlem Akgün, Fatma Gül Demirkan, Isabelle Koné-Paut, Taner Coşkuner, Veysel Çam, Merve Cansu Polat, Esra Esen, Emilio Amleto Conti, Nihal Şahin, Özge Baba and 22 more

Abstract readMulticenter Study
In one paragraph

Article in Rheumatology (Oxford, England), 2026. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.

0numbers the graph read from it
0cells of the map it votes in
1citing papers in PubMed
–field-weighted citation impact
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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

1 citing paper in PubMed.

  1. 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

32 authors.

Özlem AkgünDepartment of Pediatric Rheumatology, School of Medicine, Istanbul University, Istanbul, Turkey.ORCID 0000-0001-7216-0562
Fatma Gül DemirkanDepartment of Pediatric Rheumatology, School of Medicine, Istanbul University, Istanbul, Turkey.ORCID 0000-0001-9950-2489
Isabelle Koné-PautDepartment of Paediatric Rheumatology and CEREMAIA, Bicêtre University Paris Saclay Hospital, APHP, Le Kremlin Bicetre, France.ORCID 0000-0001-8939-5763
Taner CoşkunerDepartment of Pediatric Rheumatology, Ümraniye Research and Training Hospital, University of Health Sciences, Istanbul, Turkey.ORCID 0000-0001-7844-3387
Veysel ÇamDivision of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey.ORCID 0000-0001-6629-3967
Merve Cansu PolatDivision of Pediatric Rheumatology, Department of Pediatrics, Ankara City Hospital, University of Health Sciences, Bilkent, Ankara, Turkey.ORCID 0000-0003-3279-8435
Esra EsenDepartment of Pediatric Rheumatology, Erciyes University, Kayseri, Turkey.ORCID 0000-0003-2010-5769
Emilio Amleto ContiPediatric Immuno-Rheumatology Unit, Fondazione IRCSS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Nihal ŞahinDepartment of Pediatric Rheumatology, Kocaeli University, Izmit, Turkey.ORCID 0000-0002-2122-6952
Özge BabaPediatric Rheumatology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.ORCID 0000-0002-2442-1550
Gülşah KılbaşDepartment of Pediatric Rheumatology, Pamukkale University Faculty of Medicine, Denizli, Turkey.
Nesibe Gökçe KocamazDepartment of Pediatric Rheumatology, Ankara Etlik City Hospital, Ankara, Turkey.ORCID 0009-0007-4233-5457
Sema Nur TaşkınDepartment of Pediatric Rheumatology, Eskişehir City Hospital, Eskisehir, Turkey.ORCID 0000-0003-3400-1514
Donato RiganteDepartment of Life Sciences and Public Health, Fondazione Policlinico Universitario A. Gemelli IRCCS, Rome, Italy.ORCID 0000-0001-7032-7779
Marija JelusicDepartment of Pediatrics, University of Zagreb School of Medicine, University Hospital Centre Zagreb, Zagreb, Croatia.ORCID 0000-0002-1728-4260
Annacarin HorneDepartment of Women's and Children's Health, Karolinska Institutet Cancerforskning KI, Stockholm, Sweden.ORCID 0000-0002-2878-4463
Kübra ÖztürkDepartment of Pediatric Rheumatology, Göztepe Research and Training Hospital, Istanbul Medeniyet University, Istanbul, Turkey.ORCID 0000-0003-0466-0228
Semanur ÖzdelDepartment of Pediatric Rheumatology, Ankara Etlik City Hospital, Ankara, Turkey.ORCID 0000-0001-5602-4595
Oya KökerDivision of Pediatric Rheumatology, Department of Pediatrics, Marmara University School of Medicine, Istanbul, Turkey.ORCID 0000-0002-0101-9585
Selçuk YükselDepartment of Pediatric Rheumatology, Pamukkale University Faculty of Medicine, Denizli, Turkey.ORCID 0000-0001-9415-1640
Mukaddes KalyoncuPediatric Rheumatology, Faculty of Medicine, Karadeniz Technical University, Trabzon, Turkey.ORCID 0000-0001-5378-939X
Hafize Emine SönmezDepartment of Pediatric Rheumatology, Kocaeli University, Izmit, Turkey.ORCID 0000-0002-9186-3068
Giovanni FilocamoPediatric Immuno-Rheumatology Unit, Fondazione IRCSS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.ORCID 0000-0002-9638-6512
Ayşenur Paç KısaarslanDepartment of Pediatric Rheumatology, Erciyes University, Kayseri, Turkey.ORCID 0000-0002-1800-9922
Maria Vincenza MastroliaRheumatology Unit, ERN ReCONNET Center, Meyer Children's Hospital IRCCS, Firenze, Italy.ORCID 0000-0002-9784-3543
Banu Çelikel AcarDivision of Pediatric Rheumatology, Department of Pediatrics, Ankara City Hospital, University of Health Sciences, Bilkent, Ankara, Turkey.ORCID 0000-0002-1808-3655
Dallel BenazzouzDepartment of Paediatric Rheumatology and CEREMAIA, Bicêtre University Paris Saclay Hospital, APHP, Le Kremlin Bicetre, France.
Ezgi Deniz BatuDivision of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey.ORCID 0000-0003-1065-2363
Betül SözeriDepartment of Pediatric Rheumatology, Ümraniye Research and Training Hospital, University of Health Sciences, Istanbul, Turkey.ORCID 0000-0002-5079-5644
David SaadounFrom Sorbonne Universités Assistance Publique-Hôpitaux de Paris (AP-HP), Groupe Hospitalier Pitié- Salpêtrière, Département de Médecine Interne et Immunologie Clinique, Centre National de Références Maladies Autoimmunes Systémiques Rares, Centre National de Références Maladies Autoinflammatoires et Amylose Inflammatoire, Clinical Investigation Center in Biotherapy, and INSERM 959, Groupe Hospitalier Pitié-Salpêtrière, AP-HP, Paris, France.ORCID 0000-0003-3628-9996
Seza ÖzenDivision of Pediatric Rheumatology, Department of Pediatrics, Faculty of Medicine, Hacettepe University, Ankara, Turkey.ORCID 0000-0003-2883-7868
Nuray Aktay AyazDepartment of Pediatric Rheumatology, School of Medicine, Istanbul University, Istanbul, Turkey.ORCID 0000-0003-3594-7387

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

objectiveThis study aims to examine the indications for paediatric rheumatologists to use biologic therapies in childhood Behçet's disease (BD), as well as the efficacy, safety and remission rates of such treatments. We also compare demographic and clinical characteristics of Turkish and European cohorts.

methodsIn this multicentric retrospective study, the data of 109 paediatric BD (pedBD) patients diagnosed before 18 years of age, obtained from Pediatric Rheumatology European Society (PRES) vasculitis study group, which involves 19 centres across six countries were analysed.

resultsOf the patients, 64 were from Turkey and 45 from Europe. The primary indications for initiating biologics were ocular (40.4%), mucocutaneous (22.9%) and neurologic (17.4%) involvement. TNF-α inhibitors were used in 90.8% of cases, with adalimumab (ADA, 59 patients) and infliximab (IFX, 38 patients) being the most commonly prescribed agents. Skin involvement was more common in Turkish cohort compared with European cohort (P < 0.01), while other organ/system involvement was similar. The interval between symptom onset and diagnosis was longer in the European group. Though not significant, biological therapies were initiated earlier and had longer duration in the European cohort. Remission rates were similar in patients receiving ADA and IFX; however, patients receiving ADA experienced a faster decline in disease activity scores, but the difference was not significant (P = 0.2). Only one serious infection was reported, and no malignancy/autoimmune disease were observed.

conclusionBiologic therapies exhibit robust efficacy and an acceptable safety profile in pedBD. The study emphasizes ocular, mucocutaneous and neurological involvement as principal indications for initiating biologics and offers regional insights into therapeutic strategies.

Indexed as

Antirheumatic AgentsBehcet SyndromeBiological ProductsBiological TherapyAdalimumabAdolescentChildEuropeFemaleHumansInfliximabMaleRemission InductionRetrospective StudiesTreatment OutcomeTumor Necrosis Factor-alphaAdalimumabAntirheumatic AgentsBiological ProductsInfliximabTumor Necrosis Factor-alphaautoinflammatory conditionsBehçet’s diseasebiologic therapypaediatric/juvenile rheumatologyvasculitis

Identifiers

PMID40748718
PMCPMC13016814

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