title: Refractory pemphigus foliaceus and Behçet's disease successfully treated with tocilizumab.
abstract: Pemphigus foliaceus (PF) and Behçet's disease (BD) are immune-mediated conditions which are usually treated with corticosteroids, immunosuppressants, and, when refractory, with biologic agents. In both diseases, interleukin (IL)-6 serum levels are increased driving the immune-mediated inflammatory process. Tocilizumab is a humanized monoclonal antibody, targeting IL6-receptor, used in the treatment of rheumatoid arthritis. Besides the current indication, it has been recently administered to patients with refractory immune inflammatory diseases as an off-label treatment. Here, we report the case of a woman affected with PF and BD, who did not respond to corticosteroids, immunosuppressants, and biologic agents including adalimumab, anakinra, and infliximab. A complete, long-lasting, clinical, and serological remission was achieved only with tocilizumab. To the best of our knowledge, the association between PF and BD has never been reported. Moreover, only two cases of BD and no cases of PF treated with tocilizumab have been described to date. A literature review on the use of biologic agents on patients with PF and BD was also carried out.
authors.affiliation: Division of Rheumatology, Department of Medicine, University of Padua, Via Giustiniani 2, 35128, Padua, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Caso
authors.forename: Luca
authors.initials: L
authors.lastname: Iaccarino
authors.forename: Silvano
authors.initials: S
authors.lastname: Bettio
authors.forename: Francesca
authors.initials: F
authors.lastname: Ometto
authors.forename: Luisa
authors.initials: L
authors.lastname: Costa
authors.forename: Leonardo
authors.initials: L
authors.lastname: Punzi
authors.forename: Andrea
authors.initials: A
authors.lastname: Doria
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D001528:Behcet Syndrome / Q000150:complications / Q000628:therapy*; D018572:Disease-Free Survival; D004351:Drug Resistance; D005260:Female; D006801:Humans; D007167:Immunotherapy / Q000379:methods*; D015850:Interleukin-6 / Q000097:blood / Q000276:immunology*; D056687:Off-Label Use; D010392:Pemphigus / Q000150:complications / Q000628:therapy*; D019947:Receptors, Interleukin-6 / Q000037:antagonists & inhibitors; D012074:Remission Induction
chemical_list: D000305:Adrenal Cortex Hormones; D061067:Antibodies, Monoclonal, Humanized; D015850:Interleukin-6; D019947:Receptors, Interleukin-6; C502936:tocilizumab