European Behçet’s Recommendations Forthcoming

BARCELONA, Spain — When to use apremilast and biologics such as adalimumab and infliximab and how to deal with vascular involvement are some of the main changes to the forthcoming update of the European Alliance of Associations for Rheumatology (EULAR) Recommendations for the Management of Behçet’s Syndrome, which were presented at the organization’s 2025 Annual Meeting.

Last updated in 2018, EULAR’s draft recommendations on Behçet’s syndrome, also known as Behçet disease, or just simply Behçet’s, have undergone substantial revision, said Gülen Hatemi, MD, Cerrahpaşa Medical School, Istanbul University, Turkey.

Hatemi, one of the two co-convenors of the EULAR task force behind the revised recommendations, said there had been changes not just to the content but also to the wording of the recommendations to make them shorter to meet EULAR’s current standard operating procedures.

“The content was modified with seven recommendations only. The wording was modified with four recommendations, and we developed one new recommendation,” she said. 

Overarching Principles 

There are still five overarching principles, and the first still recognizes the “relapsing and remitting course” of Behçet’s, which could be “organ- or life-threatening,” although “disease manifestations may ameliorate over time,” Hatemi said. 

The second overarching principle, however, now states that “the goal of treatment is to prevent irreversible organ damage and to maximize health-related quality of life,” and the third states that “organ involvement should be evaluated throughout the disease course and mimickers should be ruled out with appropriate modalities.”

The fourth overarching principle states that “treatment should be individualized according to age, sex, type, and severity of organ involvement, disease duration, and patient preferences,” which was included from the prior iteration. And finally, the fifth overarching principle expands on what is necessary for optimal care: a multidisciplinary approach, patient education, shared decision-making, adherence to treatment, and lifestyle changes.

Revised Recommendations

There are now seven rather than 10 recommendations, updated according to the results of a large-scale systematic literature review and expert opinion. Out of more than 7000 published papers that had come up in the literature review, 83 articles were used for the update. 

Hatemi told Medscape Medical News that the major changes were “first, for mucocutaneous involvement refractory to colchicine, we recommended the use of apremilast or TNF [tumor necrosis factor] inhibitors.” 

This updated recommendation was based on data from two trials, she said. The first of those trials was published in the New England Journal of Medicine in 2019 and showed apremilast resulted in a greater reduction in the number of oral ulcers than placebo. The second trial was published more recently in 2024 in Annals of the Rheumatic Diseases and provides evidence for the use of infliximab and adalimumab.

A second major change concerns the management of eye involvement. Hatemi said: “For uveitis, we recommended the use of first-line monoclonal anti-TNF inhibitors, preferably infliximab for sight-threatening inflammation, and we advised against the use of glucocorticoids as monotherapy.” Immunosuppressive therapy is recommended in all patients with Behçet’s uveitis with the aim of inducing and maintaining clinical and angiographic remission. 

As for joint involvement, colchicine is the recommended first-line treatment, with immunosuppressants to be considered a back-up in recurrent or chronic cases.

“For patients with both venous and arterial involvement, we preferred infliximab over immunosuppressants, including cyclophosphamide. And we also advised the use of TNF inhibitors for long-term maintenance in order to avoid relapses,” Hatemi said. 

The one new recommendation states that “in cerebral venous sinus thrombosis with vision-threatening intracranial hypertension, surgical interventions should be given prompt consideration.”

Infliximab is also preferred for parenchymal nervous system involvement, although immunosuppressants and high-dose glucocorticoids could be used. Slow glucocorticoid tapering is advised, with immunosuppressants continued as maintenance.

As for gastrointestinal involvement, Hatemi said: “We advise the use of 5-ASA or azathioprine for mild-to-moderate cases, and monoclonal anti-TNF inhibitors for severe cases.”

Time For Treatment Harmonization

Commenting to Medscape Medical News, consultant rheumatologist Rajinder Andev, MBBS, who works at one of the three British Behçet’s Centres of Excellence, said that guidelines are “the bread and butter” on how to manage patients. 

Recommendations from EULAR and the recent British Society for Rheumatology living guidelines provide a top-line overview of which treatments have the best evidence base or expert opinion to support them. 

However, “in some of our complex cases that are refractory, that we sometimes do see, then we do have to scratch our heads and go a little bit outside the scope of the guidelines,” Andev said.

Treatment options are “still changing,” he noted, and gave an example of different infliximab preparations used to treat the various organ manifestations that can occur.

“We’re trying to get some more harmonization” across the national centers and in line with both British and European guidelines, Andev said. Although there are commonalities, “big data” from registries are perhaps needed to enable the outcomes of treatments to be compared, he suggested. 

Hatemi has disclosed receiving research support from AbbVie, Soligenix, and UCB Pharma. She has also disclosed acting as a speaker or advisor for AbbVie, Amgen, Johnson & Johnson, MSD Pharmaceuticals, Novartis, Soligenix, and UCB Pharma. Andev has reported no relevant financial relationships. 

Sara Freeman, MSc, is a freelance medical journalist based in London, UK. She has been reporting for specialist healthcare news organizations for more than 20 years.