Table 1 Patients with late onset of angioedema attacks due to deficiency of C1-INH
From: Abstracts from the 10th C1-inhibitor deficiency workshop
Group A: AAE patients | Group B: no proof for AAE | |
|---|---|---|
No of patients | 6 | 4 |
Female/male | 4/2 | 3/1 |
The age of first onset | 56 ± 6 years | 48 ± 6 years |
Other diseases | 2 patients—lymphoma, 3 patients—other npl disease (liver, kidney, breast), 1 patient—Lupus Erythematosus | 1 patient—hypertension, 1 patient—renal cysts (autoimmunity and allergy diseases excluded) |
Max. frequency of attacks | 3–4/year | 3–4/year |
The most frequent localization of the attacks | facial, laryngeal | facial, laryngeal, abdominal |
aC1INH | 0.03–0.04 g/L | 0.03–0.15 g/L |
fC1INH | 1.9–55% | 1.4–19% |
C4 | 0.01–0.03 g/L | 0.02–0.1 g/L |
C1q | 39–46 mg | – |
Treatment—positive response | 2 patients—remission of symptoms after chemotherapy of lymphoma, 4 patients—pdC1 INH | 4 patients—pdC1 INH, 1 patient rC1 INH |