Update on the treatment of Behçet’s syndrome

Esatoğlu, Sinem Nihal; Hatemi, Gülen

doi:10.1007/s11739-019-02035-1

Cited by 43 publications

(33 citation statements)

References 92 publications

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“…Due to the different phenotypes of the disease and the lack of consensual standards of care, BD treatment remains to be a challenge. The use of therapies is in many cases based on a few randomized clinical trials, singular case reports or small case series [26,27]. European League Against Rheumatism (EULAR) group has published an update of recommendations for the management of BD depending on the domain(s) affected in each patient, providing a more individualized therapeutic approach [9].…”

Section: Discussionmentioning

confidence: 99%

Apremilast in refractory orogenital ulcers and other manifestations of Behçet's disease. National multicenter study of 51 cases in clinical practice.

Atienza‐Mateo

Martín-Varillas

Gil

et al. 2020

Preprint

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Background Oral and/or genital aphthous ulcers are the most common symptoms of Behçet´s disease (BD), and are often refractory to conventional treatment. The inhibitor of phosphodiesterase-4 apremilast (APR) has demonstrated efficacy in the treatment of this manifestations. The objective of the present study was to assess the efficacy of APR in the management of refractory oral and/or genital ulcers in patients with BD. Methods National multicenter open-label observational study on BD patients with recurrent oral and/or genital ulcers. In all cases orogenital ulcers were refractory to conventional therapy. APR was given and maintained at standard dose of 30 mg twice daily. The main outcome was the achievement of oral and/or genital ulcers remission. Efficacy of APR for other clinical manifestations was also evaluated. Results We included 51 patients (35 women/16 men; mean age 44.7 ± 13.2 years). Before APR, all patients had received several systemic conventional and/or biologic drugs. APR was initiated because of refractory oral (n = 19) or genital (n = 2) aphthous ulcers or both (n = 30). Other manifestations found at APR onset were arthralgia/arthritis (n = 16), folliculitis/pseudofolliculitis (n = 14), erythema nodosum (n = 3), furunculosis (n = 2), paradoxical psoriasis induced by TNFα-inhibitors (n = 2), ileitis (n = 2), deep venous thrombosis (n = 2), leg ulcers (n = 1), erythematosus and scaly skin lesions (n = 1), fever (n = 1), unilateral anterior uveitis (n = 1) and neurobehçet (n = 1). After a mean follow-up of 8.5 ± 6.9 months, most patients had experienced improvement of orogenital ulcers and prednisone dose had been successfully reduced or discontinued. APR also yielded improvement of some non-aphthous manifestations such as the cutaneous follicular and intestinal manifestations. However, the effect on musculoskeletal manifestations was variable. Conclusion APR yielded a rapid and maintained improvement of refractory mucocutaneous ulcers of BD, even in patients refractory to several systemic drugs including biologic therapy.

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Section: Discussionmentioning

confidence: 99%

Apremilast in refractory orogenital ulcers and other manifestations of Behçet's disease. National multicenter study of 51 cases in clinical practice.

Atienza‐Mateo

Martín-Varillas

Gil

et al. 2020

Preprint

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“…Eventually, IFN α can be considered a therapeutic approach in selected cases (79). In a prospective study on patients with lower-extremity DVT, the treatment with IFN α accounted for a good recanalization and low relapse rates (80).…”

Section: Second-and Third-line Treatmentsmentioning

confidence: 99%

Treating the Different Phenotypes of Behçet's Syndrome

et al. 2019

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Behçet's syndrome (BS) is a multisystemic vasculitis, characterized by different clinical involvements, including mucocutaneous, ocular, vascular, neurological, and gastrointestinal manifestations. Based on this heterogeneity, BS can be hardly considered as a single clinical entity. Growing evidence supports that, within BS, different phenotypes, characterized by clusters of co-existing involvements, can be distinguished. Namely, three major BS phenotypes have been reported: (a) the mucocutaneous and articular phenotype, (b) the extra-parenchymal neurological and peripheral vascular phenotype, and (c) the parenchymal neurological and ocular phenotype. To date, guidelines for the management of BS have been focused on the pharmacological treatment of each specific BS manifestation. However, tailoring the treatments on patient's specific phenotype, rather than on single disease manifestation, could represent a valid strategy for a personalized therapeutic approach to BS. In the present literature review, we summarize current evidence on the pharmacological treatments for the first-, second-, and third-line treatment of the major BS phenotypes.

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“…In the manuscript "Update on the treatment of Behçet's syndrome", Hatemi reviewed the main advances in the therapeutical management of BS [45] beyond the European League Against Rheumatism (EULAR) recommendations very recently published [3].…”

Section: Treatmentsmentioning

confidence: 99%

“…OU, genital ulcers and erythema nodosum) and articular involvement should start colchicine as first-line drug. The use of disease-modifying antirheumatic drugs (DMARDs) (mainly azathioprine, AZA) should be considered in patients intolerant/resistant to colchicine [45]. The use of anti-TNF-α, interferon (IFN) α or thalidomide should be reserved to truly refractory or severe forms.…”

Section: Treatmentsmentioning

confidence: 99%

“…To date few data suggesting the optimal drugs for the management of both gastrointestinal and neurological manifestations of BS are available. AZA and anti-TNF α can be useful, respectively, even for those doubt cases for multiple sclerosis and Crohn's disease [45]. Small case series have also suggested the use of tocilizumab in refractory central nervous system involvement [65].…”

Section: Treatmentsmentioning

confidence: 99%

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