帕帕综合征:实现长期缓解的挑战
1Professor Asja Prohić, MD, PhD, Department of Dermatovenerology, Sarajevo Medical School, University Sarajevo School of Science and Technology, Sarajevo, Bosnia and Herzegovina; asjaprohic@hotmail.com.
Acta dermatovenerologica Croatica : ADC
|November 25, 2023
概括
帕帕综合征是一种遗传性自身炎症性疾病,涉及发炎性关节炎,发炎性皮肤病和. 阿达利穆马布,异丁氨酸和普雷迪尼松的多种药物疗法在稳定患者方面显示出有前途.
科学领域:
- 遗传学和免疫学 遗传学和免疫学
- 皮肤病学 皮肤病学
- 类风湿病学 类风湿病学
背景情况:
- 帕帕综合征是一种由PSTPIP1基因突变引起的自身炎症性疾病,导致异常的炎症酶激活和像IL-1β和TNF-α这样的炎症性细胞因子升高.
- 目前的治疗包括皮质类固醇和针对TNF-α或IL-1的生物药物,但数据稀缺,治疗反应不一致.
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