Prevalence: between 25 and 250 per 100,000 persons, depending on racial and geographic background Race: more prevalent in Asians and blacks Gender: female preponderance; gender ratio between 20 and 40 years; male:female, 1:9 Age: onset mainly between 20-40 y

Concordancy in twins Monozygotic: 50-60% Dizygotic: 5-10% Familial aggregation in 10% Association with the following: HLA: B7, B8, DR2, DR3, DQW1 Complement: C4A Q0

C1q or C4 deficiency Fc 7 receptor IIA low-affinity phenotype X chromosome ?

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