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.