International Rare And Severe Psoriasis Expert Network

Официальное название

International Rare And Severe Psoriasis Expert Network (IRASPEN) - A Prospective Registry With Genotype-Phenotype Correlation

Обзор

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.

Подробное описание

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.

Вмешательства

• Другое biological sampling
  In order to investigate the level of molecular pathophysiology, blood and punch biopsies will be collected of each patient. In up to two relatives per patient, 30mL blood will be collected only once.
• Другое Phenotypic description
  Phenotypic characterization of the patient's clinical features
• Другое Photography
  All affected areas will be photographed at each visit with 2-dimensional standardized photography

Первичные конечные точки

• Change in Physician Global Assessment (PGA) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]

Вторичные конечные точки (11)

• Change in Generalized Pustular Psoriasis (GPP) Area and Severity Index (GPPASI) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in Dermatology Life Quality Index (DLQI) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in EuroQol (EQ-5D) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in Work Productivity and Activity Impairment Questionnaire-General Health (WPAI-GH) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in psoriasis symptom scale (PSS) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in Disease activity Visual analogue Scale (VAS) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in Pain Visual analogue Scale (VAS) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Number of flares in the last 2 years [Срок оценки: at Baseline]
• Number of flares since the last visit [Срок оценки: 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) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]
• Change in Psoriasis Area and Severity Index (PASI) [Срок оценки: At Baseline, week 12, week 24, week 52, week 104, week 156, week 208, week 260]

Критерии участия

Критерии включения

• 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.

Критерии исключения

• 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

Критерии приведены из реестра в оригинале (на английском). Окончательную оценку соответствия проводит исследовательский центр.

Здоровые добровольцы: Нет

Дизайн исследования

Модель наблюдения

Когортное

Центры проведения

Швейцария · 2 центра

• Dermatology, University Hospital Basel — Basel
• University Hospital Zürich — Zurich

Turkey (Türkiye) · 2 центра

• Akdeniz University School of Medicine; Department of Dermatology and Venereology — Antalya
• Trakya University, Faculty of Medicine; Department of Dermatology and Venereology — Edirne

Германия · 1 центр

• Klinikum der Universität München — München

Италия · 1 центр

• Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS — Rome

Португалия · 1 центр

• Universitário do Porto — Porto

Сингапур · 1 центр

• National Skin Centre — Singapore

Идентификаторы

NCT: NCT04359394 · 2020-00425; sp18Navarini2

Первоисточники (государственные реестры)

• ClinicalTrials.gov (NIH, США)

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