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Journal Article Epublish 7 citations

PubMed: 42540035 DOI: 10.1016/j.ero.2026.02.019

Management of major organ involvement in Behçet syndrome: a systematic literature review informing the 2025 update of the EULAR recommendations.

EULAR Rheumatol Open 2(2):100152

Authors: Yesim Ozguler, Sinem Nihal Esatoglu, 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 Moots, David Saadoun, Carlo Salvarani, Petros Sfikakis, Aleksandra Stefanovic, Rosaria Talarico, Ilknur Tugal-Tutkun, Ugur Uygunoglu, Qianyu Zhao, Sofia Ramiro, Gulen Hatemi

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, CHU Dijon Bourgogne, Service de médecine interne et immunologie clinque, 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 NHS Foundation Trust, Norwich, UK.
  • Department of Medical, Surgery and Health Sciences, University of Trieste, Trieste, Italy.
  • 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, Germany.
  • Clinic for Rheumatology and Immunology, 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, UMR S959, Immunology-Immunopathology-Immunotherapy, Paris, France.
  • Rheumatology Unit, Azienda USL-IRCCS 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.
  • 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, the Netherlands.

Abstract

OBJECTIVES

The objective of this study was to evaluate and update the evidence on pharmacologic and interventional treatments for major organ involvement in Behçet syndrome (BS), in order to inform the 2025 update of the European Alliance of Associations for Rheumatology recommendations.

METHODS

A systematic literature review was conducted using 2 distinct search strategies for pharmacologic and surgical/interventional therapies, covering major databases from October 2015 to November 2024. Controlled studies comparing active interventions with placebo or another active treatment were included. If no controlled trials were available for a specific research question, uncontrolled studies or case series with at least 10 patients with BS were included.

RESULTS

Of 7128 citations, 69 studies on major organ involvement and tapering/withdrawal of immunosuppressives were included. Only 5 were randomised controlled trials (RCTs), 4 of them included patients with eye involvement, and 1 included patients with vascular or nervous system involvement. RCTs and comparative observational studies for eye involvement supported the use of monoclonal tumour necrosis factor alfa inhibitors and interferon alfa. The RCT that included patients with vascular or nervous system involvement favoured infliximab over cyclophosphamide based on complete response rates and safety. Observational data additionally supported the use of interferon alfa for venous thrombosis, whereas the role of adjunctive anticoagulation remains unclear. Noncomparative observational studies showed benefit with monoclonal tumour necrosis factor alfa inhibitors in patients with gastrointestinal involvement.

CONCLUSIONS

This systematic literature review provided evidence on the management of major organ involvement to inform the task force for developing the 2025 update of the European Alliance of Associations for Rheumatology recommendations for the management of BS.

MeSH

Behcet Syndrome, Interferon-Alpha, Necrosis, Therapeutics, Nervous System