Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra
Targeted treatment of pyoderma gangrenosum in PAPA (pyogenic arthritis, pyoderma gangrenosum and acne) syndrome with the recombinant human interleukin-1 receptor antagonist anakinra
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DOI：
10.1111/j.1365-2133.2009.09404.x
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发表时间：
2009-11-01
影响因子：
10.3
通讯作者：
Herzer, P.
中科院分区：
文献类型：
--
作者：
Brenner, M.;Ruzicka, T.;Herzer, P.
无菌性化脓性关节炎、坏疽性化脓性坏疽和痤疮三联征的首字母缩写为PAPA综合征。它是一种罕见的常染色体显性遗传性早发性疾病。坏疽性脓毒症的治疗具有挑战性，因为全身性糖皮质激素和免疫抑制治疗通常仅部分缓解。我们报告了PAPA综合征患者对重组人白细胞介素-1受体拮抗剂（rHuIL-1 Ra）阿那白滞素靶向治疗的快速和持久反应。
The triad of sterile pyogenic arthritis, pyoderma gangrenosum and acne is known by the acronym of PAPA syndrome. It is a rare autosomal dominant disease of early onset. The treatment of pyoderma gangrenosum is challenging as there is often only partial response to systemic glucocorticosteroids and immunosuppressive therapies. We report the rapid and lasting response of pyoderma gangrenosum to the targeted treatment with the recombinant human interleukin-1 receptor antagonist (rHuIL-1Ra) anakinra in a patient with PAPA syndrome.