Anti-IL5 and Other Biotherapies in Cystic Fibrosis

Study Overview
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Brief Summary
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Our project is to describe retrospectively and prospectively CF patients treated with biotherapy in French CF centers. Main objective: To describe the clinical and paraclinical course of CF patients before and after treatment with anti-IL5 and other biotherapies since 2019. Secondary objective: To describe adverse events potentially related to the biotherapies.

Detailed Description
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•Background: ABPA and asthma associated with cystic fibrosis impact the CF course with a more rapid decline in lung function. Corticosteroid therapy can be harmful and must be avoid in CF to prevent diabetes, osteoporomalacia or mycobacterium infections. Monoclonal antibodies have the marketing authorization for severe uncontrolled asthma and, up to now, some CF patients with ABPA or severe asthma and high plasma IgE levels benefit from omalizumab. Anti-Il5 agents are available since February 2019 and have demonstrated their efficacy in severe and hypereosinophilic asthma control (plasma eos.>300mmol/L). Some patients with CF who have severe asthma or ABPA are still poorly controlled despite Omalizumab with other treatments (steroids and/or azoles). Some of them have persistent hypereosinophilia suggesting a possibility to treat with antiIL5 antibodies. About 5% of patients have biotherapy treatment criteria, some have already received it, others are elective to such treatment and will receive in the future. Methods: Our project is to describe retrospectively and prospectively the clinical history of CF patients eligible for biotherapy in French CF centers. Main objective: To describe the clinical and paraclinical course of CF patients before and after treatment with anti-IL5 and other biotherapies since 2019. Secondary objective: To describe any adverse events potentially related to the biotherapies.

Official Title
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Anti-IL5 and Other Biotherapies in Cystic Fibrosis in French CF Centers : MAB-CF Cohort

Conditions
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Cystic Fibrosis

Participation Criteria
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Eligibility Criteria
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Inclusion criteria: Cystic fibrosis (2 known variants) Age ≥ 6 years Plasma hypereosinophilia ≥ 300/µl Uncontrolled ABPA or uncontrolled asthma, or failure of other biotherapy (intolerance, ineffectiveness) Exclusion criteria - Refusal to participate in this research

Ages Eligible for Study
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Minimum Age: 6 Years

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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What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Clinical evolution | Physical examination (height in centimeters, weight in kilograms, numerical scales in score, temperature in degrees, SaO2 in percentage). Defines the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis To assess the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis | 1 day | 
| Spirometry in liters or percentage evolution | Spirometry in liters or percentage Defines the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis | 1 day | 
| Biology evolution | Biology (leukocyte formula in 10^9/L, IgE tot, IgE spe, sputum microbiology) Defines the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis | 1 day | 
| concomitant therapy evolution | concomitant therapy Defines the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis | 1 day | 
| CFQR and SNOT22 evolution | CFQR and SNOT22 questionnaires in score Defines the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis | 1 day | 
| exacerbation evolution | Defines the effectiveness of anti-inflammatory biotherapy in patients with cystic fibrosis | 1 day | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Number of any adverse event reported during biotherapy treatments | Describe any adverse event potentially related to biotherapies. description of any adverse event reported during biotherapy treatments. | 1 day | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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CYSTIC FIBROSIS, BIOTHERAPY, ANTI-IL5, BENRALIZUMAB, APBA, SEVERE ASTHMA