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Table 1 Types of HAE and some associated immunoregulatory disorders

From: Membranous nephropathy in a patient with hereditary angioedema: a case report

Type Characteristics Comments Some immunological conditions associated with all types of HAE
1 Low or absent C1 esterase inhibitor activity Autosomal dominant. Constitutes 80–85% of cases Systemic lupus erythematosus, mesangiocapillary glomerulonephritis, autoimmune thyroiditis, rheumatoid arthritis, urticaria, other glomerulonephritides, Sjögren's syndrome, coagulopathies
2 Normal or raised activity of a dysfunctional C1 esterase inhibitor Autosomal dominant. Constitutes 15–20% of the cases  
3 Normal C1 esterase inhibitor level and function X linked dominant newly described in women