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