Bronchiectasis Prevalence in Patients With Primary Humoral Immunodefiency in Champagne-Ardenne Region, France

NCT06355323 | Completed

• 1 other identifier: PT22020
• Study Type: interventional
• Target: 46
• Locations: 1 country
• Sites: 1

Brief Summary

Primary humoral immunodeficiency (PHID), such as common variable immunodefiency, are the most common symptomatic primary immunodeficiency in adults, in France. Patients are more prone to infections (particularly bacterial upper and lower respiratory tract infections), auto-immunity and atopic manifestations. Morbidity and mortality in PHID are mainly linked to the presence of bronchiectasis, which can lead to infections and to chronic respiratory failure. However, bronchiectasis in these patients can be asymptomatic for a long time. There is no known predictive factors to identify patients more susceptible to develop bronchiectasis and notably, there was no link between the number of previous infectious episodes and bronchiectasis. A marked IgM deficiency and switched memory B cell deficiency might be associated with bronchiectasis. Thoracic CT-scan is recommended at PHID diagnosis but there is no guideline for follow-up, thus leading to bronchiectasis being under-diagnosis or leading to delayed diagnosis

Conditions

Primary Immunodeficiency; Common Variable Immunodeficiency; Igg Subclass Deficiency; Selective IgM Deficiency

Outcome Measures

Primary Outcomes (1)

• Thoracic CTscan

  non-contrast thin-section thoracic CT-scan, after negative urine pregnancy test for women of childbearing age, allowing to detect presence of bronchiectasis and if present the severity index (Bhalla score)

  Day 0

Secondary Outcomes (14)

• Pulmonary Function Test

  Day 0

• Pulmonary Function Test

  Day 0

• Pulmonary Function Test

  Day 0

• Pulmonary Function Test

  Day 0

• Pulmonary Function Test

  Day 0

Study Arms (1)

Patients

EXPERIMENTAL

Other: Blood test; Other: chest CT scan

Interventions

Blood test OTHER

Blood test

Patients

chest CT scan OTHER

chest CT scan

Patients

Eligibility Criteria

• Age: 18 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Adult (18-64), Older Adult (65+)

You may qualify if:

• Patients with primary humoral immunodeficiency defined as following :
• Common variable immunodeficiency : Marked decreased in IgG seric concentration \< 5 g/L and in IgA seric concentration or IgM seric concentration (-2SD), without secondary cause
• IgG subclass deficiency : marked decrease (-2SD) in at least one IgG subclass (IgG1, 2, 3 et 4) with or without IgA or IgM deficiency
• Selective IgM Deficiency : isolated decrease un IgM seric concentration \<0.3g/L, without IgG or IgA deficiency
• Adult patients
• Patients with or without previously identified pulmonary complications
• Patients agreeing to participate to the study
• Patients insured under the French social security system
• Patients\< 18 years of age
• Patients protected by law
• Patients for whom CT scan is not feasible (claustrophobia, obesity…)
• Pregnant women

Sponsors & Collaborators

• CHU de Reims: lead

Study Sites (1)

Damien JOLLY

Reims, France

Location

MeSH Terms

Conditions

Primary Immunodeficiency Diseases; Common Variable Immunodeficiency; IgG Deficiency

Interventions

Hematologic Tests

Condition Hierarchy (Ancestors)

Genetic Diseases, Inborn; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Immunologic Deficiency Syndromes; Immune System Diseases; Dysgammaglobulinemia; Blood Protein Disorders; Hematologic Diseases; Hemic and Lymphatic Diseases

Intervention Hierarchy (Ancestors)

Clinical Laboratory Techniques; Diagnostic Techniques and Procedures; Diagnosis; Investigative Techniques

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: SCREENING
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: February 24, 2023
• First Posted: April 9, 2024
• Study Start: November 28, 2022
• Primary Completion: March 11, 2024
• Study Completion: March 11, 2024
• Last Updated: December 18, 2025

Record last verified: 2025-12

Locations

FR (1)