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Apremilast in refractory orogenital ulcers and other manifestations of Behçet's disease. A national multicentre study of 51 cases in clinical practice
B. Atienza-Mateo1, J.L. Martín-Varillas2, J. Graña3, G. Espinosa4, C. Moriano5, T. Pérez-Sandoval6, M.D. García-Armario7, I. Castellví8, J.A. Román-Ivorra9, A. Olivé10, A. Ybáñez11, A. Martinez-Ferrer12, J. Narváez13, S. Romero-Yuste14, S. Ojeda15, I. Ros16, J. Loricera17, V. Calvo-Río18, S. Castañeda19, M.A. Gonzalez-Gay20, R. Blanco21
- Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
- Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
- Department of Rheumatology, Complejo Hospitalario Universitario A Coruña, Spain.
- Department of Autoimmune Diseases, Hospital Clinic, Barcelona, Spain.
- Department of Rheumatology, Complejo Asistencial Universitario de León, Spain.
- Department of Rheumatology, Complejo Asistencial Universitario de León, Spain.
- Department of Rheumatology, Hospital Lluís Alcanyís, Xàtiva, Spain.
- Department of Rheumatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
- Department of Rheumatology, Hospital La Fe, Valencia, Spain.
- Department of Rheumatology, Hospital Universitario Germans Trias i Pujol, Badalona, Spain.
- Department of Rheumatology, Hospital Universitario Doctor Peset, Valencia, Spain.
- Department of Rheumatology, Hospital Universitario Doctor Peset, Valencia, Spain.
- Department of Rheumatology, Hospital Universitario de Bellvitge, L’Hospitalet de Llobregat, Spain.
- Department of Rheumatology, Complejo Hospitalario Universitario de Pontevedra, Spain.
- Department of Rheumatology, Hospital Universitario de Gran Canaria Dr. Negrín, Las Palmas, Spain.
- Department of Rheumatology, Hospital Son Llàtzer, Mallorca, Spain.
- Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
- Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
- Department of Rheumatology, Hospital Universitario de La Princesa, IIS-Princesa, Madrid, Spain.
- Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain.
- Department of Rheumatology, Hospital Universitario Marqués de Valdecilla, IDIVAL, University of Cantabria, Santander, Spain. rblanco@humv.es
on behalf of the Spanish Collaborative Group of Refractory Behçet’s Disease
CER13778
2020 Vol.38, N°5 ,Suppl.127
PI 0069, PF 0075
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PMID: 33331268 [PubMed]
Received: 05/07/2020
Accepted : 21/09/2020
In Press: 10/12/2020
Published: 10/12/2020
Abstract
OBJECTIVES:
The objective of the present study was to assess the efficacy of apremilast (APR) in the management of refractory oral and/or genital ulcers in patients with Behçet’s disease (BD).
METHODS:
National multicentre 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 TNF-α-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 neuro Behçet (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.
CONCLUSIONS:
APR yielded a rapid and maintained improvement of refractory mucocutaneous ulcers of BD, even in patients refractory to several systemic drugs including biologic therapy.