title: Canakinumab efficacy and long-term tocilizumab administration in tumor necrosis factor receptor-associated periodic syndrome (TRAPS).
abstract: Tumor necrosis factor receptor-associated periodic syndrome (TRAPS) is an autosomal dominantly inherited autoinflammatory disease caused by mutations in the TNFRSF1A gene. Treatment is aimed at preventing acute disease attacks, improving quality of life, and preventing long-term complications such as systemic reactive amyloidosis. Biologic agents have significantly improved TRAPS management. In particular, interleukin 1 (IL-1) inhibition either with the recombinant IL-1 receptor antagonist anakinra or with the human IgG1 anti-IL-1β monoclonal antibody canakinumab has recently shown to induce a prompt and stable disease remission. Conversely, the successful experience with IL-6 inhibition is nowadays limited to a single patient. Anyway, introduction of new treatment options for patients requiring a lifelong therapy is desirable. We describe two TRAPS patients (son and father) successfully treated with canakinumab and tocilizumab, respectively. In particular, we highlight the clinical and laboratory efficacy as well as the good safety profile of tocilizumab during a 42-month follow-up period.
authors.affiliation: Department of Paediatrics, Antonio Perrino Hospital, Brindisi, Italy. latorre_francesco@virgilio.it.
authors.forename: Francesco
authors.initials: F
authors.lastname: La Torre
authors.affiliation: Department of Rheumatology, Hospital Galateo, San Cesario di Lecce, Italy.
authors.forename: Maurizio
authors.initials: M
authors.lastname: Muratore
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behcet's Disease, Policlinico Le Scotte, University of Siena, Siena, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Vitale
authors.affiliation: Department of Paediatrics, Antonio Perrino Hospital, Brindisi, Italy.
authors.forename: Fulvio
authors.initials: F
authors.lastname: Moramarco
authors.affiliation: Department of Rheumatology, Hospital Galateo, San Cesario di Lecce, Italy.
authors.forename: Laura
authors.initials: L
authors.lastname: Quarta
authors.affiliation: Research Center of Systemic Autoinflammatory Diseases and Behcet's Disease, Policlinico Le Scotte, University of Siena, Siena, Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Cantarini
mesh_terms: D000328:Adult; D000911:Antibodies, Monoclonal / Q000008:administration & dosage*; D061067:Antibodies, Monoclonal, Humanized / Q000008:administration & dosage*; D002675:Child, Preschool; D004252:DNA Mutational Analysis; D004334:Drug Administration Schedule; D005334:Fever / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D020022:Genetic Predisposition to Disease; D056660:Hereditary Autoinflammatory Diseases / Q000175:diagnosis / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage*; D008297:Male; D009154:Mutation; D010641:Phenotype; D047888:Receptors, Tumor Necrosis Factor, Type I / Q000235:genetics / Q000276:immunology; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D007166:Immunosuppressive Agents; D047888:Receptors, Tumor Necrosis Factor, Type I; C506235:TNFRSF1A protein, human; C541220:canakinumab; C502936:tocilizumab
keywords: Autoinflammatory disorders; Canakinumab; Follow-up; Therapy; Tocilizumab