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[Lupus and protein deficiencies of the classical complement pathway]
L Weiss1, F Maillet, M Kazatchkine
1Unité d'immunopathologie et INSERM U28, hôpital Broussais, Paris.
Insights
Complement deficiencies, especially C4A, are common in systemic lupus erythematosus (SLE), increasing risks for autoimmune diseases and infections. Many SLE patients have the C4 AQO silent allele, linked to anti-SSA (Ro) antibodies.
Area of Science:
- Immunology
- Genetics
- Rheumatology
Abstract:
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.