Use of TNF inhibitors in the treatment of PAPA syndrome
نویسندگان
چکیده
Introduction PAPA syndrome (pyogenic arthritis, pyoderma gangrenosum and acne syndrome) is a rare autoinflammatory disease caused by mutations in the PSTPIP1 gene. This disease is difficult to treat, but the combination of prednisone, an IL-1 inhibitor and a TNF-inhibitor has, in our experience, helped even the most severely affected patients. Treatment with anakinra appears to prevent most of the severe joint manifestations, and treatment with a TNF-inhibitor is most effective for treating and preventing the pyoderma gangrenosum lesions.
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