title: Abnormal production of tumor necrosis factor (TNF) -- alpha and clinical efficacy of the TNF inhibitor etanercept in a patient with PAPA syndrome [corrected].
abstract: We report a family with pyogenic sterile arthritis, pyoderna and acne syndrome (PAPA). The proband presented several episodes of sterile pyogenic arthritis and became unresponsive to glucocorticoids. After treatment with the tumor necrosis factor inhibitor etanercept, the disease underwent rapid and sustained clinical remission. Production of tumor necrosis factor-alpha by mononuclear cells of the proband and of the affected relatives was abnormally elevated.
authors.affiliation: Department of Medicine, Division of Rheumathology and Laboratory of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
authors.forename: Elisabetta
authors.initials: E
authors.lastname: Cortis
authors.forename: Fabrizio
authors.initials: F
authors.lastname: De Benedetti
authors.forename: Antonella
authors.initials: A
authors.lastname: Insalaco
authors.forename: Stefania
authors.initials: S
authors.lastname: Cioschi
authors.forename: Flaminia
authors.initials: F
authors.lastname: Muratori
authors.forename: Leila E
authors.initials: LE
authors.lastname: D'Urbano
authors.forename: Alberto G
authors.initials: AG
authors.lastname: Ugazio
mesh_terms: D000152:Acne Vulgaris / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D048868:Adaptor Proteins, Signal Transducing / Q000235:genetics; D000293:Adolescent; D000328:Adult; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D001168:Arthritis / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D002648:Child; D002675:Child, Preschool; D003598:Cytoskeletal Proteins / Q000235:genetics; D000068800:Etanercept; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000627:therapeutic use*; D008297:Male; D011711:Pyoderma / Q000150:complications* / Q000188:drug therapy / Q000235:genetics; D018124:Receptors, Tumor Necrosis Factor / Q000627:therapeutic use*; D013577:Syndrome; D014409:Tumor Necrosis Factor-alpha / Q000037:antagonists & inhibitors* / Q000096:biosynthesis
chemical_list: D048868:Adaptor Proteins, Signal Transducing; D000894:Anti-Inflammatory Agents, Non-Steroidal; D003598:Cytoskeletal Proteins; D007074:Immunoglobulin G; C486382:PSTPIP1 protein, human; D018124:Receptors, Tumor Necrosis Factor; D014409:Tumor Necrosis Factor-alpha; D000068800:Etanercept