Common variable immunodeficiency (CVID) and MxA-protein expression in blood leucocytes.
The underlying immunopathogenic mechanism of CVID has been suspected to involve a chronic viral infection or an autoimmune condition. However, formal proof of viral infection is lacking. Measurement of MxA-protein in leucocyte lysates is a sensitive test for evaluating the activation of the host's interferon system. Both viral infections and autoimmune diseases such as systemic lupus erythematosus (SLE) strongly induce MxA-protein in peripheral leucocytes. We therefore examined 15 patients with longlasting hypogammaglobulinaemia for MxA-protein induction in vivo: 13 patients suffered from CVID, one from hyper-IgM syndrome, and one patient had chronic B lymphocytic leukaemia associated with immunoglobulin deficiency and chronic papilloma virus infection (condylomata accuminata). Only the latter patient exhibited a strong MxA-protein expression; two CVID patients were borderline positive, and the remaining 12 patients including the hyper-IgM syndrome were MxA-protein-negative. There was no relationship between MxA expression and low CD4/CD8 ratios or increased CD8/CD57+ T cell counts, although both conditions are often observed in CVID as well as in chronic viral infections. When exposed in vitro to interferon-alpha (IFN-alpha), peripheral blood leucocytes of four MxA-negative patients were capable of producing normal amounts of MxA-protein. Taken together, these results argue against a viral or autoimmune pathogenesis of CVID.
Rump
J A
JA
Abteilung Rheumatologie, Med. Univ. Klinik, Freiburg, Germany.
Jakschiess
D
D
Walker
U
U
Schlesier
M
M
von Wussow
P
P
Peter
H H
HH
D000328:Adult; D000368:Aged; D015703:Antigens, CD / Q000096:biosynthesis; D000945:Antigens, Differentiation, T-Lymphocyte / Q000096:biosynthesis; D000998:Antiviral Agents / Q000502:physiology; D016516:CD4-CD8 Ratio; D018959:CD57 Antigens; D016827:CD8 Antigens / Q000096:biosynthesis; D017074:Common Variable Immunodeficiency / Q000378:metabolism*; D005260:Female; D019204:GTP-Binding Proteins; D006801:Humans; D007962:Leukocytes / Q000737:chemistry*; D008214:Lymphocytes / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D064547:Myxovirus Resistance Proteins; D014176:Protein Biosynthesis
D015703:Antigens, CD; D000945:Antigens, Differentiation, T-Lymphocyte; D000998:Antiviral Agents; D018959:CD57 Antigens; D016827:CD8 Antigens; C579071:MX1 protein, human; D064547:Myxovirus Resistance Proteins; D019204:GTP-Binding Proteins