[Common variable immunodeficiency disorders: Part 2. Updated clinical manifestations and therapeutic management]. / Les déficits immunitaires communs variables (DICV) : partie 2. Mise à jour clinique et thérapeutique.
Rev Med Interne
; 42(7): 473-481, 2021 Jul.
Article
em Fr
| MEDLINE
| ID: mdl-33516581
ABSTRACT
Common variable immunodeficiency disorders (CVID) are the most common symptomatic primary antibody deficiency in adults with an estimated prevalence of 1/25,000. The most frequent clinical manifestations are upper respiratory tract infections (including pneumonia, bronchitis, and sinusitis) predominantly with Streptococcus pneumoniae or H. influenzae. However, CVID are complicated in 20 to 30 % of cases of non-infectious manifestations which have been well characterized in recent years. Several complications can be observed including autoimmune, lymphoproliferative, granulomatous or cancerous manifestations involving one or more organs. These complications, mostly antibody-mediated cytopenias, are correlated with a decrease in the number of circulating switched memory B cells. Replacement therapy with polyvalent gammaglobulins has greatly improved the prognosis of these patients but it remains poor in the presence of digestive complications (especially in the case of chronic enteropathy and/or porto-sinusoidal vascular disease), pulmonary complications (bronchiectasis and/or granulomatous lymphocytic interstitial lung disease) and when progression to lymphoma. Much progress is still to be made, in particular on the therapeutic management of non-infectious complications which should benefit in the future from targeted treatments based on knowledge of genetics and immunology.
Palavras-chave
Autoimmune cytopenias; Common variable immunodeficiency disorders; Cytopénies auto-immunes; Déficit immunitaire commun variable; Granulomatose; Granulomatous disease; Immunoglobulins replacement therapy; Maladie vasculaire porto-sinusoïdale; Porto-sinusoidal vascular disease; Substitution par immunoglobulines
Texto completo:
1
Base de dados:
MEDLINE
Assunto principal:
Pneumonia
/
Infecções Respiratórias
/
Bronquiectasia
/
Imunodeficiência de Variável Comum
Tipo de estudo:
Diagnostic_studies
/
Prognostic_studies
/
Risk_factors_studies
Limite:
Humans
Idioma:
Fr
Revista:
Rev Med Interne
Ano de publicação:
2021
Tipo de documento:
Article