Acquired C1INH deficiency often closely resembles HAE-C1INH, except that patients develop angioedema later in life (usually after the age of 40), lack a positive family history of angioedema, often show low plasma levels of C1q, and frequently have (or will develop) an underlying disease (especially lymphoproliferative malignancies, monoclonal gammopathies, or autoimmune diseases such as systemic lupus erythematosus) or C1INH autoantibodies. Here, SERPING1 is linked to systemic lupus erythematosus.