CLOUD-R HAE REGISTRY (CloudRHAE)

International Registry of Isolated Angioedema

Angioedema is a disease characterized by the appearance of self-limiting edema that last 1-5 days and affect the subcutaneous tissue and/or gastrointestinal and oropharyngeal mucosa. In this last location edema can be lethal causing asphyxia, in all other cases full recovery is complete. Attacks can appear as part of the syndrome urticaria angioedema and can be of allergic origin. But there is a group of non-allergic angioedema that occur without hives and can be either hereditary or acquired. These angioedema are identified in some cases on the basis of etiology, in others are identified by the mediator and an overall classification of these forms of angioedema was published in 2014 as a result of a consent conference organized by the proponents of this registry. Being these forms of angioedema rare, there are not significative case studies inclusive of a high number of patients. This registry aims to collect in a single place a high number of subjects with recurring angioedema and without urticaria.

Study Overview

• Status: Recruiting
• Conditions: ANGIOEDEMA
• Intervention / Treatment: Behavioral: angioedema

Detailed Description

The Grenoble center is the registry coordinator at the national level. It will contract directly with the Cloud R Company and will be in charge of coordinating the associated centers. It will therefore send the necessary documents for the good execution of the study, will ask each center to respect the current French regulatory, will organize and finance the set-up of the register in the associated centers.

The coordinating center will also centralize requests for data, publications or creation of additional eCRF pages of associated centers. It will be able to organize a national scientific committee every 6 months. It will thus be the link between the French centers, the scientific committee of the registry and the company Cloud R.

The coordinating center will also be responsible for enter and reliability of patient data from it center, as well as the associated centers.

Role of associated centers Associated Centers will be responsible for capturing and data reliability of their patients in eCRF, they will also create a personal account for each patient so that it can download the application "cloud R HAE carnet de suivi".

Investigators referent patient will have validated at each visit or after contact with the patient crisis returned to the patient via it electronic diary.

Associated Centers will keep informed twice a year the coordinator center of the state of inclusions in the register.

• Study Type: Observational
• Enrollment (Anticipated): 1000

Contacts and Locations

• Study Contact: Name: laurence bouilelt, MD, PhD; Phone Number: +33476765565; Email: lbouillet@chu-grenoble.fr
• Study Contact Backup: Name: Isabelle Boccon-Gibod; Phone Number: +3347676767640; Email: iboccon-gibod@chu-grenoble.fr

Study Locations

• France
  • Isere
    • Grenoble, Isere, France, 38043
      • Recruiting
      • University Hospital Grenoble
      • Contact: Laurence Bouillet, MD, PhD; Phone Number: +33476765565; Email: lbouillet@chu-grenoble.fr

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: ADULT; OLDER_ADULT; CHILD
• Accepts Healthy Volunteers: No
• Genders Eligible for Study: All

• Sampling Method: Non-Probability Sample

Study Population

Any patient with isolated angioedema without associated wheals

Description

Inclusion Criteria:

• Any patient with isolated angioedema without associated wheals.
• Patients with hereditary angioedema with or without C1 deficiency.
• Major patient with consent (signature)
• Minor patient whose parents / legal guardians have given their consent (signature)

Exclusion Criteria:

• Patient unable to give consent.
• Wheals with angioedema

Study Plan

How is the study designed?

Number of groups / cohorts

5

Cohorts and Interventions

Group / Cohort	Intervention / Treatment
Hereditary angioedema type I/II; Hereditary angioedema with C1Inh deficiency	Behavioral: angioedema; electronic tracking book
Acquired angioedema; Angioedema with acquired C1Inh deficiency	Behavioral: angioedema; electronic tracking book
Drug induced angioedema; Angioedema associated with ACEi used	Behavioral: angioedema; electronic tracking book
Mast cell induced angioedema; Spontaneous mast cell induced isolated angioedema	Behavioral: angioedema; electronic tracking book
Hereditary angioedema with nC1Inh; Hereditary angioedema with normal C1Inh and with F12, PLG, ANGPT2 mutations	Behavioral: angioedema; electronic tracking book

What is the study measuring?

Primary Outcome Measures

Outcome Measure	Measure Description	Time Frame
Demographic patient data	Sexe and age of patient	5 years
Clinical characteristics	summary of patient visit report	5 years
Comorbidity	additional diseases	5 years
Angioedema diagnosis	type of angiodema	5 years
Angioedema diagnosis	position of genetic mutation if applicable	5 years
Angioedema diagnosis	biology marker : C1inh, C4, C1q, anti C1inh	5 years
Angioedema diagnosis	family history	5 years
Duration and severity of acute angioedema events	start date	5 years
Duration and severity of acute angioedema events	localization	5 years
Duration and severity of acute angioedema events	time before treatment	5 years
Duration and severity of acute angioedema events	time to resolution	5 years
Duration and severity of acute angioedema events	need access to hospital	5 years
Treatments used in managing the acute angioedema event	name	5 years
Treatments used in managing the acute angioedema event	dose	5 years
Treatments used in managing the acute angioedema event	date and time of acute treatment	5 years
Treatments used in the prophylaxis of angioedema	name	5 years
Treatments used in the prophylaxis of angioedema	dose	5 years
Treatments used in the prophylaxis of angioedema	start date	5 years

Collaborators and Investigators

• Sponsor: University Hospital, Grenoble
• Investigators: Principal Investigator: laurence bouillet, University Hospital, Grenoble

Study record dates

Study Major Dates

• Study Start (ACTUAL): September 1, 2018
• Primary Completion (ANTICIPATED): September 1, 2028
• Study Completion (ANTICIPATED): September 1, 2028

Study Registration Dates

• First Submitted: January 8, 2019
• First Submitted That Met QC Criteria: February 15, 2019
• First Posted (ACTUAL): February 19, 2019

Study Record Updates

• Last Update Posted (ACTUAL): November 1, 2021
• Last Update Submitted That Met QC Criteria: October 29, 2021
• Last Verified: October 1, 2021

More Information

Terms related to this study

• Additional Relevant MeSH Terms: Cardiovascular Diseases; Vascular Diseases; Skin Diseases; Immune System Diseases; Hypersensitivity, Immediate; Skin Diseases, Vascular; Hypersensitivity; Urticaria; Angioedema
• Other Study ID Numbers: 38RC17.187

Plan for Individual participant data (IPD)

• Plan to Share Individual Participant Data (IPD)?: NO

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No