title: Inhibition of interleukin-1 by canakinumab as a successful mono-drug strategy for the treatment of refractory Behçet's disease: a case series.
abstract: Recommendations related to ocular, mucosal and cutaneous involvement of Behçet's disease (BD) are mainly evidence-based, but in cases of vascular, neurological and gastrointestinal involvement there are no guidelines to define the best treatment strategy. We report three adult patients with BD, who received an interleukin-1β inhibitor by subcutaneous injections, canakinumab (at the dosage of 150 mg every 6 weeks), after failure shown by corticosteroids and different combinations of immunosuppressant agents. The prompt and sustained clinical efficacy demonstrated by canakinumab as a monotherapy supports the opportunity of using this specific anti-interleukin-1β agent as a valid therapeutic option for resistant or refractory BD. Open trials and observational studies should be performed to test canakinumab efficacy on a larger number of patients. The most appropriate dosage and intervals between administrations should be decided according to the individual patient, severity or recurrence of clinical manifestations and major organ involvement.
authors.affiliation: Research Center of Systemic Autoimmune and Autoinflammatory Diseases, Rheumatology Unit, Policlinico Le Scotte, University of Siena, Siena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.forename: Donato
authors.initials: D
authors.lastname: Rigante
authors.forename: Francesco
authors.initials: F
authors.lastname: Caso
authors.forename: Maria Giuseppina
authors.initials: MG
authors.lastname: Brizi
authors.forename: Mauro
authors.initials: M
authors.lastname: Galeazzi
authors.forename: Luisa
authors.initials: L
authors.lastname: Costa
authors.forename: Rossella
authors.initials: R
authors.lastname: Franceschini
authors.forename: Orso Maria
authors.initials: OM
authors.lastname: Lucherini
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D061067:Antibodies, Monoclonal, Humanized; D001528:Behcet Syndrome / Q000175:diagnosis* / Q000188:drug therapy*; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007279:Injections, Subcutaneous; D053583:Interleukin-1beta / Q000008:administration & dosage / Q000037:antagonists & inhibitors*; D008297:Male; D008875:Middle Aged; D012008:Recurrence; D012494:Sampling Studies; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D053583:Interleukin-1beta; C541220:canakinumab