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Its heightened expression has been intimately linked to the initiation and severity of diverse autoimmune diseases, spanning rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis (SSc), inflammatory bowel disease, autoimmune diabetes, and even SARS-CoV-2 infection

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One of the sequenced families included a female patient who has been investigated for several years in search of pathophysiological explanation for her HAE C1-INH-nl with typical clinical manifestations