TY - JOUR
T1 - Common variable immunodeficiency
T2 - Clinical and immunological features of 248 patients
AU - Cunningham-Rundles, Charlotte
AU - Bodian, Carol
PY - 1999/7
Y1 - 1999/7
N2 - Common variable immunodeficiency (CVI) is a primary immunodeficiency disease characterized by reduced serum immunoglobulins and heterogeneous clinical features. In these studies we describe the clinical and immunological status of 248 consecutively referred CVI patients of age range 3-79 years who have been followed for a period of 1-25 years. The median age at the time of onset of symptoms was 23 years for males and 28 years for females; the mean age at which the diagnosis of CVI was made was 29 years for males and 33 years for females. Forty percent of patients had impaired T cell proliferation to one or more mitogens; lymphocyte transformation to mitogens was directly related to the level of the serum IgG. Females at all ages had higher levels of serum IgM than males. Survival 20 years after diagnosis of CVI was 64% for males and 67% for females, compared to the expected 92% population survival for males and 94% for females. Parameters associated with mortality in this period were lower levels of serum IgG, poorer T cell responses to phytohemagglutinin, and, particularly, a lower percentage of peripheral B cells (P < 0.006).
AB - Common variable immunodeficiency (CVI) is a primary immunodeficiency disease characterized by reduced serum immunoglobulins and heterogeneous clinical features. In these studies we describe the clinical and immunological status of 248 consecutively referred CVI patients of age range 3-79 years who have been followed for a period of 1-25 years. The median age at the time of onset of symptoms was 23 years for males and 28 years for females; the mean age at which the diagnosis of CVI was made was 29 years for males and 33 years for females. Forty percent of patients had impaired T cell proliferation to one or more mitogens; lymphocyte transformation to mitogens was directly related to the level of the serum IgG. Females at all ages had higher levels of serum IgM than males. Survival 20 years after diagnosis of CVI was 64% for males and 67% for females, compared to the expected 92% population survival for males and 94% for females. Parameters associated with mortality in this period were lower levels of serum IgG, poorer T cell responses to phytohemagglutinin, and, particularly, a lower percentage of peripheral B cells (P < 0.006).
KW - CVI
KW - CVID
KW - Common variable immunodeficiency
KW - Intravenous immunoglobulin
KW - Survival
UR - http://www.scopus.com/inward/record.url?scp=0032976666&partnerID=8YFLogxK
U2 - 10.1006/clim.1999.4725
DO - 10.1006/clim.1999.4725
M3 - Article
C2 - 10413651
AN - SCOPUS:0032976666
SN - 1521-6616
VL - 92
SP - 34
EP - 48
JO - Clinical Immunology
JF - Clinical Immunology
IS - 1
ER -