title: Rituximab effectiveness in a patient with juvenile systemic lupus erythematosus complicated with acquired Von Willebrand syndrome.
abstract: The acquired von Willebrand Syndrome (AvWS) is a bleeding disorder with laboratory findings similar to those of the congenital form. Its presentation is more common in adults than in children and is mainly associated with lymphoproliferative and myeloproliferative diseases and rarely with autoimmune diseases including systemic lupus erythematosus (SLE). Here, we report the case of a girl with SLE and AvWS with mucosal bleeding and low plasma levels of von Willebrand factor and factor VIII (FVIII) with failure to respond to treatment with first- and second-line therapies. The patient finally responded to rituximab. To our knowledge this is the first reported case of SLE associated with AvWS, that responded to rituximab.
authors.affiliation: 1Hospital General Centro Medico Nacional La Raza IMSS - Reumatologia Pediatrica, Mexico, D.F.
authors.forename: A R Torres
authors.initials: AR
authors.lastname: Jimenez
authors.forename: E Solis
authors.initials: ES
authors.lastname: Vallejo
authors.forename: M Zeferino
authors.initials: MZ
authors.lastname: Cruz
authors.forename: A Cespedes
authors.initials: AC
authors.lastname: Cruz
authors.forename: J V Ramirez
authors.initials: JV
authors.lastname: Miramontes
authors.forename: B Sanchez
authors.initials: BS
authors.lastname: Jara
mesh_terms: D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use*; D002648:Child; D005169:Factor VIII / Q000378:metabolism; D005260:Female; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy*; D000069283:Rituximab; D013577:Syndrome; D016896:Treatment Outcome; D014842:von Willebrand Diseases / Q000150:complications / Q000188:drug therapy*; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D014841:von Willebrand Factor; D000069283:Rituximab; D005169:Factor VIII
keywords: Systemic lupus erythematosus; acquired von Willebrand Syndrome; rituximab