[Lupus and protein deficiencies of the classical complement pathway].
Deficiencies in proteins of the classic complement pathway are particularly frequent in patients with autoimmune diseases, notably systemic lupus erythematosus (SLE). The C4 component is a polymorphous glucoprotein coded by two closely linked genes, C4A and C4B, located within the HLA complex. C4, and in particular the C4A isotype plays a major role in maintaining immune complexes in solution. Fifty percent of patients with SLE are homozygous or heterozygous to the silent allele C4 AQO. Hereditary CE deficiency is often complicated by lupus-related diseases which may be associated with repeated infections. The biological particularity of SLE associated with complement protein deficiencies is the frequency of anti-SSA (Ro) antibodies.
Weiss
L
L
Unité d'immunopathologie et INSERM U28, hôpital Broussais, Paris.
Maillet
F
F
Kazatchkine
M
M
D003175:Complement C2 / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D003181:Complement C4 / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D003171:Complement Pathway, Classical / Q000235:genetics*; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications* / Q000175:diagnosis
D003175:Complement C2; D003181:Complement C4
Lupus et déficits en protéines de la voie classique du complément.