RESUMEN
Activated phosphoinositide 3-kinase delta syndrome (APDS) is an inborn error of immunity with heterogeneous clinical manifestations of infections, immune dysregulation, autoimmunity; lymphoproliferation; and malignancy. Immune complex-mediated vasculitides have not yet been described in APDS patients. Here we offer a case series of three patients with APDS who have refractory IgA vasculitis (also called Henoch-Schönlein purpura), a form of immune complex-mediated vasculitis that activates complement and attracts neutrophils, macrophages and eosinophils to cause local tissue injury. Leniolisib is an inhibitor of PI3K p110δ and an FDA-approved treatment for APDS. IgA vasculitis resolved upon treatment with leniolisib. Patients with immune dysregulation including IgA vasculitis should be screened for APDS.
Asunto(s)
Arteritis de Células Gigantes , Granulomatosis con Poliangitis , Vasculitis por IgA , Síndrome Mucocutáneo Linfonodular , Poliarteritis Nudosa , Piridinas , Pirimidinas , Humanos , Complejo Antígeno-Anticuerpo , Fosfatidilinositol 3-Quinasa/uso terapéutico , Fosfatidilinositol 3-QuinasasRESUMEN
A nonrandomized, retrospective comparison of Staphylococcus aureus bacteremia between an academic hospital setting (n=53) and a community hospital setting (n=245) within a single healthcare system was performed. Despite infectious disease consultations, S. aureus bacteremia management recommendations based on Infectious Diseases Society of America (IDSA) guidelines were not followed as closely in the community hospital setting. The community hospital setting requires management standardization for patients with S. aureus bacteremia. Infect Control Hosp Epidemiol 2017;38:740-742.