Abnormal production of tumor necrosis factor (TNF) -- alpha and clinical efficacy of the TNF inhibitor etanercept in a patient with PAPA syndrome [corrected].
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.
Cortis
Elisabetta
E
Department of Medicine, Division of Rheumathology and Laboratory of Rheumatology, IRCCS Ospedale Pediatrico Bambino Gesù, Rome, Italy.
De Benedetti
Fabrizio
F
Insalaco
Antonella
A
Cioschi
Stefania
S
Muratori
Flaminia
F
D'Urbano
Leila E
LE
Ugazio
Alberto G
AG
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
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