Treatment for hereditary angioedema with normal C1 ‐INH and specific mutations in the F12 gene (HAE‐FXII)
We report our experience treating HAE‐FXII with discontinuation of potential trigger factors and drug therapies. The study included 72 patients with HAE‐FXII. Potential triggers included estrogen‐containing oral contraceptives (eOC), hormonal replacement therapy, or angiotensin‐converting enzyme inhibitors. Drug treatment comprised plasma‐derived C1 inhibitor (pdC1‐INH) for acute swelling attacks and progestins, tranexamic acid, and danazol for the prevention of attacks. Discontinuation of eOC was effective in 25 (89.3%) of 28 women and led to a reduction in the number of attacks (about 90%). After ending hormonal replacement therapy, three of eight women became symptom‐free. Three women with exacerbation of HAE‐FXII during intake of quinapril or enalapril had no further HAE‐FXII attacks after discontinuation of those drugs. Eleven women were treated with pdC1‐INH for 143 facial attacks. The duration of the treated facial attacks (mean: 26.6 h; SD: 10.1 h) was significantly shorter than that of the previous 88 untreated facial attacks in the same women (mean: 64.1 h; SD: 28.0 h; P < 0.01). The mean reduction in attack frequency was 99.8% under progestins after discontinuing eOC (16 women), 93.8% under tranexamic acid (four women), and 100% under danazol (three women). For patients with HAE‐FXII, various treatment options are available which completely or at least partially reduce the number or duration of attacks.
Source: Allergy - Category: Allergy & Immunology Authors: K. Bork, K. Wulff, G. Witzke, J. Hardt Tags: Brief Communication Source Type: research