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Summary of the conduct of the study. Homozygosity of the 28‐bp deletion rs9332736 in C2 is associated with monogenic lupus. Heterozygous individuals are not at risk of SLE or primary SS if they have 2 or more copies of C4A, whereas individuals heterozygous for rs9332736 who have 1 copy of C4A are at substantial risk of both SLE and primary SS. OR = odds ratio (see Figure 3 for other definitions).
