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阿那白滞素治疗化脓性关节炎、痤疮、坏疽性脓皮病和化脓性汗腺炎（PAPASH）谱系障碍：一例报告及文献综述

Anakinra in Pyogenic Arthritis, Acne, Pyoderma Gangrenosum, and Suppurative Hidradenitis (PAPASH) Spectrum Disorder: A Case Report and Literature Review.

作者信息

Ahmad Anam, Moore Terry

机构信息

Internal Medicine, St. Luke's Hospital, Chesterfield, USA.

Internal Medicine, Saint Louis University School of Medicine, St. Louis, USA.

出版信息

Cureus. 2024 Jun 12;16(6):e62247. doi: 10.7759/cureus.62247. eCollection 2024 Jun.

DOI:10.7759/cureus.62247
PMID:39006661
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11244945/
Abstract

Pyogenic arthritis, acne, pyoderma gangrenosum, and suppurative hidradenitis (PAPASH); pyogenic arthritis, pyoderma gangrenosum (PG), and acne; PG, acne, hidradenitis suppurativa; and PG, acne, spondylarthritis (PASS) are all part of a spectrum of autoinflammatory disorders that share similar pathogenesis. They are related to various mutations in the proline-serine-threonine phosphatase interacting protein 1, leading to dysregulation of the innate immune system and overproduction of interleukin (IL)-1, IL-17, and IL-23 and tumor necrosis factor (TNF)-α. Targeting these cytokines with biologics plays an important role in treatment. Here, we are describing the case of a young male with PAPASH syndrome who was treated with TNF-α and IL-1 inhibitor.

摘要

化脓性关节炎、痤疮、坏疽性脓皮病和化脓性汗腺炎（PAPASH）；化脓性关节炎、坏疽性脓皮病（PG）和痤疮；PG、痤疮、化脓性汗腺炎；以及PG、痤疮、脊柱关节炎（PASS）均属于一系列具有相似发病机制的自身炎症性疾病。它们与脯氨酸 - 丝氨酸 - 苏氨酸磷酸酶相互作用蛋白1的各种突变有关，导致先天性免疫系统失调以及白细胞介素（IL）-1、IL-17、IL-23和肿瘤坏死因子（TNF）-α过度产生。使用生物制剂靶向这些细胞因子在治疗中起着重要作用。在此，我们描述了一名患有PAPASH综合征的年轻男性患者，该患者接受了TNF-α和IL-1抑制剂治疗的病例。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e65b/11244945/72b6958055fd/cureus-0016-00000062247-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e65b/11244945/2c4de77c093a/cureus-0016-00000062247-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e65b/11244945/72b6958055fd/cureus-0016-00000062247-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e65b/11244945/2c4de77c093a/cureus-0016-00000062247-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e65b/11244945/72b6958055fd/cureus-0016-00000062247-i02.jpg

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