engleski

RESPIRATORY MANIFESTATIONS OF COMMON VARIABLE IMMUNODEFICIENCY (CVID)

Primary humoral immunodeficiencies are diseases with alterations in the antibody response and consecutively with an increased rate of infections with encapsulated bacterial organisms. This group includes diseases with exclusively humoral deficiency and diseases that show defect of both humoral and cellular immunity. Although usually considered as a pediatric disease, almost half of all cases appear during adolescence or adulthood. In addition, increased life expectancy due to immunoglobulin replacement therapy and the late diagnosis of milder variants than previously known have changed perception of those diseases. Common variable immunodeficiency (CVID) is the most frequent clinically relevant primary immunodeficiency and an example of immunodeficiency that appears in adults. It is characterized by decreased serum immunoglobulin levels, inability to make specific antibodies, and consequently recurrent bacterial sinopulmonary infections. Because of irregular cellular immunity the patients with CVID have also increased risk to develop a broad spectrum of associated autoimmune disorders, polyclonal lymphocytic proliferation and neoplasia. Some of those non-infective complications can affect respiratory system and cause bronchitis, bronchiectasis, hilar and mediastinal lymphadenopathy, lymphocytic interstitial pneumonia (LIP), obliterative lymphocytic bronchiolitis, and granulomatous lymphocytic interstitial disease (GLILD). The care of those patients requires lifelong clinical vigilance and appropriate management of complications. Recent clinical and laboratory studies have expanded our recognizing and understanding of CVID which is necessary to improve treatment and quality of life of those patients.

Common variable immunodeficiency, lung affection

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