[Respiratory manifestations in primary immunodeficiencies]

The respiratory infections are the most common presentations and leading cause of morbidity and mortality in primary immunodeficiencies. The pathogen or spectrum of pathogens may be characteristic for the underlying primary immunodeficiency, and that knowledge plays an important role in the empirical treatment or prevention of the infections, or, conversely, the identification of an unusual pathogen may raise the suspicion on an immunodeficiency. Apart from the acute respiratory infections, chronic lung diseases are also common complications. The recurrent infections result in bronchiectasis, an irreversible structural damage of the respiratory tract, that frequently serve as locus for subsequent infections. The other group of chronic pulmonary complications are the interstitial lung diseases, which seem to be independent from infections and can be regarded as the pulmonary manifestation of the immune dysregulation. It is important to identify those patients with immunodeficiency who are at increased risk of secondary lung complications and to regularly screen this group for the early detection of the pulmonary complications. Orv Hetil. 2018; 159(49): 2043-2049.