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Ann Rheum Dis. 2018 Jun;77(6):808-818. doi: 10.1136/annrheumdis-2018-213225. Epub 2018 Apr 6.

2018 update of the EULAR recommendations for the management of Behçet's syndrome.

Author information

1
Division of Rheumatology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
2
Musculoskeletal Statistics Unit, The Parker Institute, Bispebjerg and Frederiksberg Hospital & Department of Rheumatology, Odense University Hospital, Copenhagen, Denmark.
3
Department of Dermatology, Catholic Kwandong University International St. Mary's Hospital, Incheon, Korea.
4
Department of Ophthalmology, Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France.
5
Division of Gastroenterology, Department of Internal Medicine, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
6
Centre for Clinical and Diagnostic Oral Sciences, Barts and the London School of Medicine and Dentistry, Queen Mary University of London, and the London Behçet's Centre, Barts Health London, London, UK.
7
Department of Vascular Surgery, Pitié-Salpêtrière Hospital, Sorbonne University, Paris, France.
8
Division of Rheumatology, Department of Internal Medicine, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
9
Department of Rheumatology, Immunology and Nephrology, Asklepios Clinic Altona, Hamburg, Germany.
10
Rheumatology Institute of Lucania (IRel) and the Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, Potenza and Matera, Italy.
11
Department of Internal Medicine, Hospital Saint-Louis, Paris, France.
12
National Behcet's Syndrome Centre of Excellence, Aintree University Hospital, Liverpool, UK.
13
Institute for Haematopathology Hamburg, Hamburg, Germany.
14
Department of Inflammation-Immunopathology-Biotherapy, Sorbonne Universités, UPMC Univ Paris 06, Paris, France.
15
INSERM, Paris, France.
16
CNRS, Paris, France.
17
Department of Internal Medicine and Clinical Immunology, Centre de Référence des Maladies Auto-Immunes et Systémiques Rares, Centre de Référence des Maladies Auto-Inflammatoires, AP-HP, Groupe Hospitalier Pitié-Salpêtrière, Paris, France.
18
Division of Rheumatology, Azienda USL-IRCCS di Reggio Emilia, University of Modena and Reggio Emilia, Modena and Reggio Emilia, Italy.
19
Patient Research Partner, Catania, Italy.
20
First Department of Propaedeutic and Internal Medicine & Rheumatology Unit, National Kapodistrian University of Athens Medical School, Athens, Greece.
21
Department of Neurology, Cerrahpasa Medical School, Istanbul University, Istanbul, Turkey.
22
Department of Ophthalmology, St. Thomas' Hospital, London, UK.
23
Department of Ophthalmology, Istanbul Faculty of Medicine, Istanbul University, Istanbul, Turkey.
24
Patient Research Partner, Member of the UK Behcet's Syndrome Society and Director of Behcets International, London, UK.
25
Rheumatology Institute of Lucania (IRel) and the Rheumatology Department of Lucania, San Carlo Hospital of Potenza and Madonna delle Grazie Hospital of Matera, and the Basilicata Ricerca Biomedica (BRB) Foundation, Potenza and Matera, Italy.

Abstract

Several new treatment modalities with different mechanisms of action have been studied in patients with Behçet's syndrome (BS). The aim of the current effort was to update the recommendations in the light of these new data under the auspices of the European League Against Rheumatism (EULAR) Standing Committee for Clinical Affairs. A task force was formed that included BS experts from different specialties including internal medicine, rheumatology, ophthalmology, dermatology, neurology, gastroenterology, oral health medicine and vascular surgery, along with a methodologist, a health professional, two patients and two fellows in charge of the systematic literature search. Research questions were determined using a Delphi approach. EULAR standardised operating procedures was used as the framework. Results of the systematic literature review were presented to the task force during a meeting. The former recommendations were modified or new recommendations were formed after thorough discussions followed by voting. The recommendations on the medical management of mucocutaneous, joint, eye, vascular, neurological and gastrointestinal involvement of BS were modified; five overarching principles and a new recommendation about the surgical management of vascular involvement were added. These updated, evidence-based recommendations are intended to help physicians caring for patients with BS. They also attempt to highlight the shortcomings of the available clinical research with the aim of proposing an agenda for further research priorities.

KEYWORDS:

anti-tnf; behcet’s disease; treatment

Conflict of interest statement

Competing interests: BB received consultant fees from Santen, AbbVie, Allergan, Xoma and research grants from Novartis and Bayer. ITT received honoraria from Servier, AbbVie and Allergan. GH received research grants from Celgene, honoraria from Pfizer and speaker’s fees from Abbvie, MSD and UCB.

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