Pulmonary Manifestations of Primary Humoral Deficiencies.
Can Respir J
; 2022: 7140919, 2022.
Article
em En
| MEDLINE
| ID: mdl-35440951
ABSTRACT
Primary immunodeficiencies are a group of conditions characterized by developmental or functional alterations in the immune system caused by hereditary genetic defects. Primary immunodeficiencies may affect either the innate or the adaptive (humoral and cellular) immune system. Pulmonary complications in primary humoral deficiencies are frequent and varied and are associated with high morbidity and mortality rates. The types of complications include bronchiectasis secondary to recurrent respiratory infections and interstitial pulmonary involvement, which can be associated with autoimmune cytopenias, lymphoproliferation, and a range of immunological manifestations. Early detection is key to timely management. Immunoglobulin replacement therapy reduces the severity of disease, the frequency of exacerbations, and hospital admissions in some primary humoral deficiencies. Therefore, the presence of pulmonary disease with concomitant infectious and/or autoimmune complications should raise suspicion of primary humoral deficiencies and warrants a request for immunoglobulin determination in blood. Once diagnosis is confirmed; early immunoglobulin replacement therapy will improve the course of the disease. Further studies are needed to better understand the pathogenesis of pulmonary disease related to primary humoral deficiencies and favor the development of targeted therapies that improve the prognosis of patients.
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Bronquiectasia
/
Síndromes de Imunodeficiência
/
Pneumopatias
Tipo de estudo:
Screening_studies
Limite:
Humans
Idioma:
En
Revista:
Can Respir J
Ano de publicação:
2022
Tipo de documento:
Article
País de afiliação:
Espanha