International Rare And Severe Psoriasis Expert Network (IRASPEN) - A Prospective Registry With Genotype-Phenotype Correlation
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 180
- 试验地点
- 8
- 主要终点
- Change in Physician Global Assessment (PGA)
研究概览
简要总结
This registry is a prospective observational study in order to describe primarily the natural course of PP subtypes and to gain detailed information about their phenotype.
详细描述
This project is to describe the natural course of disease in different subtypes of PP. The network builds on a static registry that was based on a one-time clinical characterization of PP patients in Europe (ERASPEN). The International Rare and Severe Psoriasis Expert Network (IRASPEN) already has multiple clinicians involved who have successfully characterized and included their patients in ERASPEN. IRASPEN addresses the question of temporal evolution of clinical features and is actually a non-interventional prospective registry that aims to describe the clinical course and responses to already established treatments of a large number of PP patients over a period of 5 years. The data collection with this registry will give insight on the natural course of PP disease revealing the burden of disease including frequency and severity of flares and the role of therapeutic interventions.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 6 Months 至 —（Child, Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Written informed consent of the patient or legal proxy in the registry
- •Diagnosis of PP confirmed by a dermatologist in the participant. The type of PP can be any one of PPP, GPP/Acute Generalized Exanthematous Pustulosis (AGEP), ACH or a mixed phenotype, according to the judgment of the investigator
- •GPP: Primary, sterile, macroscopically visible epidermal pustules on non-acral Skin with or without systemic Inflammation; with or without plaque psoriasis; either relapsing (>1 episode) or persistent (>3 months)
- •PPP: Primary, persistent (>3 months), sterile, macroscopically visible epidermal pustules on palms and/or soles with or without plaque psoriasis
- •ACH: Primary, persistent (>3 months), sterile, macroscopically visible epidermal pustules affecting the nail apparatus with or without plaque psoriasis
- •At the timepoint of inclusion, the participant must have had active pustulation with either white, yellow or brown pustules within six month before baseline. Active postulation at baseline is not mandatory for inclusion.
- •Sufficient language skills (in the languages which the patient information and the consent form is available) for the informed consent to participate
- •Patients of all ancestries and skin pigment type can be included
- •Direct non-affected adult (>18 years old) relatives of the participant (up to two, namely mother, father, sibling) with the purpose to provide DNA for family trio sequencing analysis. The patient is not excluded from the study if no relatives are included.
排除标准
- •Any medical or psychological condition in the treating physician's opinion which may prevent the patient in registry participation for the next 5 years
- •Lack of informed consent for registry participation
结局指标
主要结局
Change in Physician Global Assessment (PGA)
时间窗: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260
modified PGA (physician's assessment of psoriatic lesions) scoring the erythema, pustules, and scaling of all GPP or PPP lesions from 0 to 4. Each component is graded separately, the average is calculated, and the final PGA is determined from this composite score. A lower score indicates a lesser severity, with 0 being clear and 1 being almost clear.
次要结局
- Number of flares in the last 2 years(at Baseline)
- Change in Work Productivity and Activity Impairment Questionnaire-General Health (WPAI-GH)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in Pain Visual analogue Scale (VAS)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in Generalized Pustular Psoriasis (GPP) Area and Severity Index (GPPASI)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in Dermatology Life Quality Index (DLQI)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in EuroQol (EQ-5D)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in psoriasis symptom scale (PSS)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in Disease activity Visual analogue Scale (VAS)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Number of flares since the last visit(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in Palmoplantar Pustulosis (PPP) Area and Severity Index (PPPASI)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)
- Change in Psoriasis Area and Severity Index (PASI)(At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260)