Molecule

C1q

Central autoantigen and complement initiator

Expression change
Depleted (consumed)
Evidence level
established
Targeted by
Anti-C1q autoantibodies (pathogenic)

Role in pathogenesis

C1q is the primary autoantigen in HUVS. Anti-C1q autoantibodies bind to the collagen-like region of C1q, forming immune complexes that activate the classical complement pathway. C1q levels are markedly reduced due to consumption. Low C1q is the most sensitive serologic marker.

Sources (4)

DetailsWisnieski JJ et al. (1989) Serum IgG antibodies to C1q in hypocomplementemic urticarial vasculitis syndromePubMed
DetailsWisnieski JJ et al. (1992) Comparison of autoantibodies to the collagen-like region of C1q in hypocomplementemic urticarial vasculitis syndrome and systemic lupus erythematosusPubMed
DetailsMarzano AV et al. (2022) Urticarial vasculitis: Clinical and laboratory findings with a particular emphasis on differential diagnosisDOI
DetailsWisnieski JJ et al. (1995) Hypocomplementemic urticarial vasculitis syndrome. Clinical and serologic findings in 18 patientsDOI