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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