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E primary complement defects such as complete deficiency of the early components of classical complement pathway genes (C1q, C1r/s, C2, C4A and C4B) are strongly associated with increased susceptibility to SLE [61?64]. The occurrence of a lupus-like syndrome or SLE has been demonstrated in 90 of homozygous C1q deficient cases, in more than 50 of cases with C1s/C1r deficiencies, in 10 to 30 of C

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