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 | Â |