Lung diseases in adult patients with primary immunodeficiencies with defects of antibody production according to real clinical practice

Introduction. The problem of lung diseases in patients with primary defects in antibody production has not been sufficiently studied, especially depending on the climatic, geographical and demographic conditions of real clinical practice.

Aim. To study the structure of lung disorders in adult patients with primary antibodies defects in the Middle Urals in real clinical practice.

Materials and methods. Register of adult patients with primary immunodeficiencies (PID) were created in 2013 in the Sverdlovsk region. Now it contains 209 people. The main group of the register is patients with primary antibodies defects (PAD, n = 143, 68.4%: agammaglobulinemia (AGG, n = 11, common variable immune deficiency (CVID, n = 37), PIK3-Kinase deficiency (n = 3), Selective IgA deficiency (SD IgA, n = 92). The diagnosis of PID was established on the criteria for the Russian Association of Allergists and Clinical Immunologists and European Societies of Immunodeficencies, in some cases it has a genetic confirmation. We used medical history of patients, radiological, functional studies to establish lung lesions. We occurred immunological examination for all PID patients.

Results. Repeated pneumonia were observed in all patients with AGG and CVID, especially in the onset of PID. Patients also had diseases such as bronchiectasis (up to 37.6% of patients), chronic obstructive lung disease (up to 70.3% of patients), bronchial asthma (only SD IgA), interstitial lung disease (only CVID).

Conclusion. According to our data, in the Middle Urals, lung diseases, especially pneumonia and bronchiectasis, are the most common clinical manifestations in patients with DA. Analysis of immunoglobulins' level is necessary in patients with repeated pneumonia, bronchiectasis and interstitial lung disease, bronchial asthma and early onset of chronic obstructive lung disease without smoking status.

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