Clinical StudiesIdiopathic nonhistaminergic angioedema
Section snippets
Methods
We studied 25 patients (15 men and 10 women; ages 16 to 77 years; median age 42 years) with recurrent symptoms of angioedema (three or more attacks per year for 1 year or longer) without urticaria. These symptoms were not prevented by H1 blockers (hydroxyzine up to 100 mg per day). Known causes of angioedema (Figure 1 ) were excluded in all patients by obtaining a clinical history with detailed information about personal and familial allergies; association of the angioedema with potential
Results
The median age at onset of symptoms in patients with idiopathic nonhistaminergic angioedema was 35 years (range 8 to 66), and the median duration of symptoms at the time of our first visit was 6 years (range 1 to 25). The average frequency of angioedema attacks was >12 attacks per year for 16 patients, six to 11 attacks per year for 6 patients, and one to five attacks per year for 3 patients. There was a family history of angioedema in 4 patients: in two families, 2 brothers were affected (in
Discussion
As consultants for patients with suspected C1-inhibitor deficiency, we have examined >500 patients with angioedema of varied origin. Among them, we identified a group of 25 patients with recurrent attacks of angioedema that were not associated with urticaria or with previous history of allergy. No cause of angioedema was detectable in these patients, and drugs that block the H1 receptor did not reduce the severity of attacks nor prevent their recurrence. Therefore, we have called this syndrome
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