背景:泛发性脓疱型银屑病(3GPP)是一种慢性,罕见,以及潜在威胁生命的皮肤状况,其特征是耀斑,包括广泛的无菌脓疱和全身性炎症。该疾病的稀有性和异质性都使得GPP分类和临床标准的标准化具有挑战性。在2022年批准spesolimab(IL-36R抗体)之前,在美国或欧洲没有批准的用于GPP耀斑的治疗方法。对3GPP的治疗相当于使用批准用于治疗斑块状牛皮癣的药物的标签外使用。我们的目的是描述社会人口统计学,临床特征,和治疗模式的病人在西班牙的事。
方法:非介入,描述性,描述性多中心,在西班牙被诊断为3GPP的患者的回顾性图表回顾。
结果:纳入56例患者(50%为女性),诊断时的平均(SD)年龄为53.7(20.5),平均(SD)随访时间为3.7(3.1)年。在80%的患者中,3GPP诊断与耀斑相关,67.3%的患者有已知的风险因素[如先前诊断或斑块型银屑病家族史,合并症,吸烟或压力。高血压和斑块状银屑病是最常见的合并症(各占44.6%)。每个患者年的3GPP耀斑数量为0.55,(范围0-4)平均(SD)体表面积受累为21.3%(19.1)。3GPP耀斑最常见的表现是脓疱(88.5%),红斑(76.9%)和鳞屑(76.9%)。此外,65.4%的患者有斑块状银屑病,53.8%有不明皮肤损伤,30.8%的人经历过疼痛。用于3GPP耀斑的治疗是标签外的常规全身性药物(75%),主要是皮质类固醇,环孢菌素和阿维A。在耀斑之间的时期,56.5%的患者使用了标签外生物制剂。在学习期间,9例(16.1%)患者出现至少一种并发症,其中5人需要住院治疗。
结论:这是第一个在西班牙的多中心研究患者。大多数病人都在五十多岁,有斑块状银屑病的个人或家族史,压力,吸烟和广泛的合并症和并发症。即使每位患者/年的耀斑数量为0.55,患者之间也存在差异。标签外常规系统和标签外生物制剂均用于耀斑管理,而没有明确的治疗模式。
BACKGROUND: Generalized pustular psoriasis (GPP) is a chronic, rare, and potentially life-threatening skin condition characterized by flares comprising widespread sterile pustules and systemic inflammation. Both the rarity and heterogeneity of the disease have made GPP classification and standardization of clinical criteria challenging. Before the approval of spesolimab (IL-36R antibody) in 2022, there were no approved treatments in the USA or Europe for GPP flares. Treatment for GPP has amounted to off-label use of medicines approved to treat plaque psoriasis. Our aim was to describe the sociodemographics, clinical characteristics, and treatment patterns of patients with GPP in Spain.
METHODS: Non-interventional, descriptive, multi-center, retrospective chart review of patients diagnosed with GPP in Spain.
RESULTS: 56 patients (50% women) were included, with a mean (standard deviation, SD) age at diagnosis of 53.7 (20.5) and a mean (SD) time of follow-up of 3.7 (3.1) years. In 80% of patients, GPP diagnosis was associated with a flare and 67.3% had known risk factors for GPP (such as previous diagnosis or family history of plaque psoriasis, comorbidities, smoking or stress). Hypertension and plaque psoriasis were the most frequent comorbidities (44.6% each). The number of GPP flares per patient-year was 0.55 with (range 0-4) a mean (SD) body surface area involvement of 21.3% (19.1). The most frequent manifestations of GPP flares were pustules (88.5%), erythema (76.9%), and scaling (76.9%). Additionally, 65.4% of patients had plaque psoriasis, 53.8% had unspecified skin lesions, and 30.8% experienced pain. The treatments used for GPP flares were off-label conventional systemic drugs (75%), mostly corticosteroids, cyclosporine, and acitretin. In the periods between flares, off-label biologics were used in 56.5% of patients. During the study period, 9 patients (16.1%) had at least one complication and 5 of them required hospitalization.
CONCLUSIONS: This is the first multicenter study in Spanish GPP patients. Most patients were in their fifties, with personal or family history of plaque psoriasis, stress, smoking and a wide range of comorbidities and complications. Even though the number of flares per patient/year was 0.55, there was variability between patients. Both off-label conventional systemics and off-label biologics were used for flare management without a clear treatment pattern.