CLOUD-R HAE REGISTRY

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

Official Title
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International Registry of Isolated Angioedema

Conditions
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ANGIOEDEMA

Intervention / Treatment
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* Behavioral: angioedema


Participation Criteria
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Eligibility Criteria
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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
Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Hereditary angioedema type I/II<br>Hereditary angioedema with C1Inh deficiency | Behavioral: angioedema<br>* electronic tracking book<br>|
| Acquired angioedema<br>Angioedema with acquired C1Inh deficiency | Behavioral: angioedema<br>* electronic tracking book<br>|
| Drug induced angioedema<br>Angioedema associated with ACEi used | Behavioral: angioedema<br>* electronic tracking book<br>|
| Mast cell induced angioedema<br>Spontaneous mast cell induced isolated angioedema | Behavioral: angioedema<br>* electronic tracking book<br>|
| Hereditary angioedema with nC1Inh<br>Hereditary angioedema with normal C1Inh and with F12, PLG, ANGPT2 mutations | Behavioral: angioedema<br>* electronic tracking book<br>|

What is the study measuring?
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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 |