Treatment of hereditary angioneurotic edema with tranexamic acid and cinnarizine

Abstract

Hereditary angioneurotic edema (HANE) is a disease with an autosomal dominant inheritance, in which the complement either has a low esterase inhibitor of the first component (C1 INH) or else this is biochemically inactive. Even the fourth (C4) and second (C2) components of the complement are reduced (1,8). The clinical features are paroxysmal edema of subcutaneous tissues and mucosa, abdominal pain and laryngeal edema, which may prove fatal. Some patients have red streaks and rings on their skin in conjunction with swellings. Less frequent symptoms are urine retention due to edema of the urinary tract and cerebral symptoms such as severe headache, aphasia, and hemiplegia. Six out of 7 Finnish patients suffering from hereditary angioneurotic edema were helped during attacks, by treatment with tranexamic acid (AMCA, Cyklokapron, Kabi) in doses of 1.5 g 3 times daily, follow up time 3-25 months. 3 of these patients also had continuous AMCA treatment, in the course of which 2 remained nearly symptom free on a dosage of 1 g 2-3 times daily. Even the third one had shorter and milder attacks. One patient, however, had to stop taking AMCA after 6 weeks' treatment, because of fatigue and nausea. Follow up time for the others was 9-11 months. For comparative purposes, 3 patients were given continuous treatment with an antihistamine, cinnarizine in a dosage of 20-30 mg daily. Two of the patients were helped by it, one becoming nearly symptom free and the other having fewer and milder attacks; follow up time 9-10 months.