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The interesting report by Karagianni P et al on the finding of increased DNA methylation of H19 locus imprinting control region in saliva samples of Sjögren\'s syndrome patients correlating with low complement C4 levels [1], may offer insights into how C4 level may be regulated in serpinopathies such as C1-inhibitor deficiency. An undetectable or low C4 level in patients with severe angioedema is a feature of C1-inhibitor deficiency (hereditary angioedema (HAE) type I with low to absent function and antigenic levels; HAE type II with point mutations in SERPING1 gene that affect the reactive centre loop affecting protein function only).
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Clinical_and_experimental_immunology
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Epigenetic alterations on C1-inhibitor expression may influence hereditary angioedema attack frequency and C4 levels.
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