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Journal Article Epublish 7 citations

PubMed: 42540112 DOI: 10.1016/j.ero.2026.02.017

Management of mucocutaneous and joint involvement of Behçet's syndrome: a systematic literature review informing the 2025 update of the EULAR recommendations.

EULAR Rheumatol Open 2(2):100150

Authors: Sinem Nihal Esatoglu, Yesim Ozguler, Gunnar Tomasson, Louise Falzon, Stephane Barete, Alessandra Bettiol, Bahram Bodaghi, Vladimira Boyadzhieva, Luca Cantarini, Aykut Ferhat Celik, Omar Dhrif, Georgina Ducker, Giacomo Emmi, Ahmet Gül, Jörg Henes, Ina Koetter, Martin Krusche, Giuseppe Lopalco, Robert J Moots, David Saadoun, Carlo Salvarani, Petros Sfikakis, Aleksandra Stefanovic, Rosaria Talarico, Ilknur Tugal-Tutkun, Ugur Uygunoglu, Qianyu Zhao, Gulen Hatemi, Sofia Ramiro

Affiliations

  • Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University - Cerrahpasa, Istanbul, Türkiye.
  • Behçet's Disease Research Center, Istanbul University - Cerrahpasa, Istanbul, Türkiye.
  • Centre of Public Health Sciences, Faculty of Medicine, School of Health Sciences, University of Iceland, Reykjavik, Iceland.
  • Internal Medicine and Emergency Services, Landspitali-The National University Hospital of Iceland, Reykjavik, Iceland.
  • School of Medicine and Population Health, University of Sheffield, Sheffield, UK.
  • Department of Dermatology, Pitié-Salpêtrière University Hospital, Sorbonne University, Paris, France.
  • Department of Experimental and Clinical Biomedical Sciences "Mario Serio", University of Florence, Florence, Italy.
  • Department of Ophthalmology, Reference Center for Rare Diseases, Pitié-Salpêtrière Hospital, APHP-Sorbonne University, Paris, France.
  • Department of Rheumatology, Clinic of Rheumatology, University Hospital St. Ivan Rilski, Medical University of Sofia, Sofia, Bulgaria.
  • Department of Medical Sciences, Surgery and Neurosciences, Research Center of Systemic Autoinflammatory Diseases and Behçet's Disease Clinic, University of Siena, Siena, Italy.
  • Azienda Ospedaliero-Universitaria Senese [European Reference Network (ERN) for Rare Immunodeficiency, Autoinflammatory and Autoimmune Diseases (RITA) Center] Siena, Italy.
  • Division of Gastroenterology, Department of Internal Medicine, Istanbul University-Cerrahpaşa, Istanbul, Türkiye.
  • Université Bourgogne Europe, Centre Hospitalier Universitaire Dijon Bourgogne, Service de médecine interne et immunologie clinque, Institut National de la Santé et de la Recherche Médicale (INSERM) U1098, Dijon, France.
  • Faculty of Medicine of Tunis, University of Tunis El Manar, Tunis, Tunisia.
  • Vasculitis Specialist Nurse, Department of Rheumatology, Norfolk and Norwich University Hospitals National Health Service (NHS) Foundation Trust, Norwich, UK.
  • Department of Medical, Surgery and Health Sciences, University of Trieste, and Clinical Medicine and Rheumatology Unit, Cattinara University Hospital, Trieste, Italy.
  • Centre for Inflammatory Diseases, Monash University Department of Medicine, Monash Medical Centre, Melbourne, Australia.
  • Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Türkiye.
  • Department for Internal Medicine II (Hematology, Oncology, Rheumatology and Clinical Immunology), University Hospital Tuebingen, Tuebingen, Germany.
  • Division of Rheumatology and Inflammatory Systemic Diseases, III. Department of Medicine, University Medical Center Eppendorf, Hamburg and Clinic for Rheumatology and Immunology at Auenlandclinic Bad Bramstedt, Germany.
  • Division of Rheumatology and Systemic Inflammatory Diseases, III. Department of Medicine, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
  • Department of Precision and Regenerative Medicine and Ionian Area, University of Bari, Bari, Italy.
  • Faculty of Health, Care and Medicine, Edge Hill University, Ormskirk, UK.
  • Liverpool University Hospitals NHS Foundation Trust, Aintree University Hospital, Liverpool, UK.
  • Department of Internal Medicine and Clinical Immunology, Sorbonne Universités, Assistance Publique-Hôpitaux de Paris, Pitié-Salpêtrière University Hospital, Centre national de références Maladies Autoimmunes et systémiques rares, National Reference Centre for Rare Autoinflammatory Diseases and Inflammatory Amyloidosis, INSERM, Unité Mixte de Recherche (UMR) S959, Immunology-Immunopathology-Immunotherapy, Paris, France.
  • Rheumatology Unit, Azienda Unità Sanitaria Locale - Istituto di Ricovero e Cura a Carattere Scientifico di Reggio Emilia, Reggio Emilia, Italy.
  • Department of Surgery, Medicine, Dentistry and Morphological Sciences, with Interest in Transplants, Oncology and Regenerative Medicine, University of Modena and Reggio Emilia, Modena, Italy.
  • First Department of Propaedeutic Internal Medicine, Medical School, Laiko General Hospital, National and Kapodistrian University of Athens, Athens, Greece.
  • Patient Research Partner, Belgrade, Serbia.
  • Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy.
  • Azienda Ospedaliero Universitaria Pisana, Pisa, Italy.
  • Uveitis Service, Eye Protection Foundation, Bayrampasa Eye Hospital, Istanbul, Türkiye.
  • Department of Neurology, Cerrahpaşa Medical Faculty, Istanbul University-Cerrahpaşa, Istanbul, Türkiye.
  • Patient Research Partner, Paris, France.
  • Department of Rheumatology, Leiden University Medical Center, Leiden and Zuyderland Medical Center, Heerlen, Netherlands.

Abstract

OBJECTIVES

We aimed to update the evidence for the management of mucocutaneous and joint involvement of Behçet's syndrome (BS) to inform the 2025 update of European Alliance of Associations for Rheumatology (EULAR) recommendations.

METHODS

A systematic literature review was conducted to evaluate the benefits and harms of therapeutic interventions in patients with BS. All comparative studies were included, and in the absence of controlled studies for a specific intervention, noncomparative studies were also considered. We presented here the summary of the outcomes for mucocutaneous and joint involvement, overall disease activity and health-related quality of life (HRQoL).

RESULTS

Among the 7128 articles, 16 studies (7 randomised controlled trials [RCTs]) reported on mucocutaneous involvement, joint involvement, overall disease activity, or HRQoL. Apremilast was superior to placebo for oral ulcers in an RCT with low risk of bias (RoB). Few patients experienced genital ulcers, skin lesions, or arthritis during the 12 weeks. In the first head-to-head RCT with low RoB comparing adalimumab with infliximab, both drugs provided similar response rates for mucocutaneous manifestations, with a more rapid response observed with adalimumab. In other head-to-head RCTs, all with low RoB except for 1 with some concerns, the efficacy of immunosuppressive agents was confounded by concomitant glucocorticoid use for major organ involvement and by the small number of patients with active mucocutaneous manifestations.

CONCLUSIONS

This systematic review supported the use of apremilast and tumour necrosis factor alpha inhibitors in patients with refractory mucocutaneous involvement and provided evidence for updating the 2025 EULAR Recommendations for the management of mucocutaneous and joint involvement of BS.

MeSH

Quality Of Life, Behcet Syndrome, Ulcer, Adalimumab