Anti-C1q IgG autoantibodies

Pathogenic autoantibody

Expression
Elevated (90-100% of patients)
Evidence level
established

Role in pathogenesis

IgG autoantibodies that bind the collagen-like region of C1q via their F(ab')2 fragments. Present in 90-100% of HUVS patients. Unlike SLE anti-C1q antibodies, HUVS antibodies can bind reduced/denatured C1q. Drive complement activation and immune complex deposition in vessel walls.