Elewski Boni, Lebwohl Mark G
Department of Dermatology, University of Alabama at Birmingham, Birmingham, AL, USA.
Icahn School of Medicine at Mount Sinai, New York, NY, USA.
Clin Cosmet Investig Dermatol. 2024 Nov 4;17:2487-2493. doi: 10.2147/CCID.S501061. eCollection 2024.
Generalized pustular psoriasis (GPP) is a rare, chronic, inflammatory skin disease characterized by persistent symptoms and sudden flares of painful, sterile pustules, and may be accompanied by systemic inflammation. Ongoing symptoms of GPP can have a serious impact on patient quality of life, morbidity, and mortality, and severe flares may be life-threatening if left untreated. Guidelines have been developed for the treatment of GPP flares; however, health care professionals and patients are lacking guidance on the management of long-term, persistent symptoms of GPP. Spesolimab is the only FDA-approved treatment for GPP and is approved for use in adults and pediatric patients aged 12 years or older and weighing at least 40 kg. Spesolimab recently gained FDA approval as a subcutaneous injection to treat GPP when patients are not experiencing a flare. In this podcast episode, we discuss what is known about the chronic disease burden of GPP and how persistent symptoms affect quality of life when patients are not experiencing a flare. We address the need for treatment guidelines for chronic GPP and discuss the results of the EFFISAYIL 2 clinical trial, which led to the approval of the subcutaneous formulation of spesolimab to treat GPP when patients are not experiencing a flare. Finally, we discuss what can be done to improve the treatment of patients with chronic GPP, both while experiencing a flare, and while living with persistent symptoms.
泛发性脓疱型银屑病(GPP)是一种罕见的慢性炎症性皮肤病,其特征为症状持续存在且疼痛性无菌脓疱突然发作,并可能伴有全身炎症。GPP的持续症状会对患者的生活质量、发病率和死亡率产生严重影响,如果不治疗,严重发作可能会危及生命。已经制定了GPP发作的治疗指南;然而,医疗保健专业人员和患者在GPP长期持续症状的管理方面缺乏指导。司库奇尤单抗是唯一获得美国食品药品监督管理局(FDA)批准用于治疗GPP的药物,被批准用于12岁及以上、体重至少40公斤的成人和儿科患者。司库奇尤单抗最近获得FDA批准,可作为皮下注射剂,用于治疗未发作的GPP患者。在本期播客中,我们讨论了已知的GPP慢性病负担,以及在患者未发作时持续症状如何影响生活质量。我们阐述了制定慢性GPP治疗指南的必要性,并讨论了EFFISAYIL 2临床试验的结果,该试验促成了皮下注射用司库奇尤单抗获批,用于治疗未发作的GPP患者。最后,我们讨论了在GPP患者发作期间以及长期存在症状时,如何改善其治疗。