Apremilast in Refractory Behçet's Syndrome: A Multicenter Observational Study

Matheus Vieira, Solène Buffier, Mathieu Vautier, Alexandre Le Joncour, Yvan Jamilloux, Mathieu Gerfaud-Valentin, Laurence Bouillet, Estibaliz Lazaro, Stéphane Barete, Laurent Misery, Delphine Gobert, Tiphaine Goulenok, Olivier Fain, Karim Sacre, Pascal Sève, Patrice Cacoub, Cloé Comarmond, David Saadoun
Frontiers in Immunology 2020, 11: 626792

Objective: Mucocutaneous and joint disorders are the most common manifestations in Behçet's syndrome (BS) and are frequently clustered in the so-called minor forms of BS. There remains a need for safe and effective treatment for joint lesions in BS. We report the long-term safety and effectiveness of apremilast in refractory joint and mucocutaneous manifestations of BS.

Methods: French nationwide multicenter study including 50 BS patients with either active joint and/or mucocutaneous manifestations resistant to colchicine and/or DMARDs. Patients received apremilast 30 mg twice a day. Primary effectiveness endpoint was the proportion of patients with complete response (CR) of articular symptoms at month 6 (M6), defined as resolution of inflammatory arthralgia and arthritis, with joint count equal to zero.

Results: At inclusion, the median tender and swollen joint count was of 4 [2-6] and 2 [1-2], respectively. The proportion of CR in joint disease at M6 was 65% (n = 15/23), and 17% (n = 4/23) were partial responders. CR of oral and genital ulcers, and pseudofolliculitis at M6 was 73% (n = 24/33), 94% (n = 16/17) and 71% (n = 10/14), respectively. The overall response at M6 was 74% for the entire cohort and 70% for the mucocutaneous-articular cluster (n = 27). The median Behçet's syndrome activity score significantly decreased during study period [50 (40-60) vs. 20 (0-40); p < 0.0001]. After a median follow-up of 11 [6-13] months, 27 (54%) patients were still on apremilast. Reasons for apremilast withdrawal included adverse events (n = 15, 30%) and treatment failure (n = 8, 16%). Thirty-three (66%) patients experienced adverse events, mostly diarrhea (n = 19, 38%), nausea (n = 17, 34%) and headache (n = 16, 32%).

Conclusion: Apremilast seems effective in BS-related articular disease refractory to colchicine and DMARDs. Discontinuation rates were significantly higher than that reported in clinical trials.

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Christoph Haller

Gelenke, nach 6 Monaten 2/3 besser

OZT Stopp war häufiger als in den klinischen VorStudien


Christoph Haller

AM: real life, Nationwide.
Alle auf Apremilast, bei therapierefraktärität.
Colchizin bei fast allen, DMARDs 50%. 1/3 PDN.

OTZ teil in Kombination gegeben (Colchizin, MTX).

OTZ auf Gelenke: 2/3 in kompletter Gele ks Remission (!)

46% hatten OTZ gestoppt (NW)


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