Common variable immunodeficiency: more than one disease? Three case reports.

Citation
L. Mouthon et al., Common variable immunodeficiency: more than one disease? Three case reports., ANN MED IN, 150(4), 1999, pp. 275-282
Citations number
34
Categorie Soggetti
General & Internal Medicine
Journal title
ANNALES DE MEDECINE INTERNE
ISSN journal
0003410X → ACNP
Volume
150
Issue
4
Year of publication
1999
Pages
275 - 282
Database
ISI
SICI code
0003-410X(199906)150:4<275:CVIMTO>2.0.ZU;2-A
Abstract
Common variable immunodeficiency (CVID) is a major antibody-deficiency synd rome, associated with increased risk of bacterial infection, as well as aut oimmune and granulomatous disease. The clinical and immunological features are heterogeneous. This heterogeneity is expressed by the case reports of t hree selected patients. These observations will be discussed, with referenc e to a recent classification of CVID distinguishing four different clinical entities: i) CVID presenting with clinical and immunological features of X -linked agammaglobulinemia; ii) CVID presenting with clinical and immunolog ical features of X-linked hyper-IgM syndrome; iii) CVID associated with sys temic granulomatous disease; and iiii) CVID associated with autoimmune mani festations.