Zmiany w układzie oddechowym występujące u pacjentów z pierwotnymi niedoborami odporności o typie humoralnym

Dominika Drąg, Urszula Szuba, *Agnieszka Oronowicz-Jaśkowiak, Magdalena Chojnowska, Wojciech Feleszko

Summary
Primary antibody deficiencies are the most common primary immunodeficiencies. The diseases affect primarily pediatric patients, and their consequences extend to the whole lifespan of the individual. This group of diseases includes, among others, common variable immunodeficiency, X-linked agammaglobulinemia, selective IgA deficiency, IgM deficiency, and IgG subclass deficiency. Due to the relative rarity of these disorders and symptoms that are mostly non-specific, the diagnosis is often delayed, even by a few years. The pulmonary and respiratory changes are relatively common and include not only infectious diseases, with pneumonia being one of the most frequent complication, but also bronchiectasis, chronic lung disease, bronchopulmonary dysplasia, granulomatous-lymphocytic interstitial lung disease, and various lung tumors. The aim of this review was to present the respiratory signs and symptoms in patients with primary antibody deficiencies and discuss the therapeutic options. It must be underlined that the presence of diagnosis delay can frequently worsen the outcomes of the treatment, therefore these patients should be diagnosed as soon as it is possible.

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