Supplementary Material for: Clinical Characteristics and Outcomes of Generalized Pustular Psoriasis (GPP) Flares

Background: Generalized pustular psoriasis (GPP) is a rare, neutrophilic skin disease that can become life-threatening if flares are untreated. There are limited data describing the characteristics and clinical course of GPP disease flares with current treatment options. Objective: To describe the characteristics and outcomes of GPP flares using historical medical information from patients enrolled in the Effisayil™ 1 trial. Methods: Investigators collected retrospective medical data characterizing patients’ GPP flares prior to clinical trial enrollment. Data on overall historical flares were collected, as well as information on patients’ typical, most severe, and longest past flares. This included data on systemic symptoms, flare duration, treatment, hospitalization, and time to clearance of skin lesions. Results: In this cohort (N=53), patients with GPP experienced a mean of 3.4 flares per year. Flares were painful, associated with systemic symptoms, and often triggered by stress, infections, or treatment withdrawal. Resolution of flares was longer than 3 weeks in 57.1%, 71.0%, and 85.7% of documented (or identified) typical, most severe, and longest flares, respectively. GPP flares led to patient hospitalization in 35.1%, 74.2%, and 64.3% of patients for their typical, most severe, and longest flares, respectively. For the majority of patients, pustules took up to 2 weeks to clear for a typical flare and 3–8 weeks to clear for the most severe and longest flares. Conclusion: Our findings highlight that current treatment options are slow to control GPP flares and provide context for assessing the efficacy of new therapeutic strategies in patients with a GPP flare.

背景：泛发性脓疱型银屑病（Generalized pustular psoriasis, GPP）是一种罕见的嗜中性皮肤病，若发作未得到及时治疗可危及生命。目前针对采用现有治疗方案的GPP发作的特征与临床病程，相关研究数据较为匮乏。目的：本研究旨在利用Effisayil™ 1临床试验入组患者的既往医疗信息，描述GPP发作的特征与临床转归。方法：研究者收集了临床试验入组前患者GPP发作的回顾性医疗数据，涵盖患者总体既往发作的相关信息，以及典型发作、最严重发作与最长持续发作的详细资料，包括全身症状、发作时长、治疗方案、住院情况以及皮肤病变清除所需时间等。结果：本研究队列（N=53）中，GPP患者年均发作次数均值为3.4次。发作多伴随疼痛与全身症状，常由应激、感染或停药诱发。在已记录的典型发作、最严重发作与最长持续发作中，分别有57.1%、71.0%与85.7%的发作持续时长超过3周。针对典型发作、最严重发作与最长持续发作，GPP发作导致患者住院的比例分别为35.1%、74.2%与64.3%。对于多数患者，典型发作的脓疱清除时间不超过2周，而最严重发作与最长持续发作的脓疱清除时间则需3~8周。结论：本研究结果显示，现有治疗方案对GPP发作的控制速度较为缓慢，同时为评估新型治疗策略在GPP发作患者中的疗效提供了参考依据。