International Rare And Severe Psoriasis Expert Network

Official title

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

Overview

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.

Detailed description

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.

Interventions

• Other 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.
• Other Phenotypic description
  Phenotypic characterization of the patient's clinical features
• Other Photography
  All affected areas will be photographed at each visit with 2-dimensional standardized photography

Primary outcome measures

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

Secondary outcome measures (11)

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

Eligibility criteria

Inclusion criteria

• 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.

Exclusion criteria

• 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

Criteria are shown verbatim from the registry (in English). Final eligibility is always assessed by the study center.

Healthy volunteers: No

Study design

Observational model

Cohort

Study locations

Switzerland · 2 centers

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

Turkey (Türkiye) · 2 centers

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

Germany · 1 center

• Klinikum der Universität München — München

Italy · 1 center

• Fondazione Policlinico Universitario "Agostino Gemelli" IRCCS — Rome

Portugal · 1 center

• Universitário do Porto — Porto

Singapore · 1 center

• National Skin Centre — Singapore

Identifiers

NCT: NCT04359394 · 2020-00425; sp18Navarini2

Primary sources (government registries)

• ClinicalTrials.gov (US NIH)

View this study on ClinicalTrials.gov ↗