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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

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