Allen P. Kaplan, MD
Kaplan AP. Acquired C1 Esterase Inhibitor Deficiency. Ann Intern Med. 2000;133:838. doi: 10.7326/0003-4819-133-10-200011210-00023
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Published: Ann Intern Med. 2000;133(10):838.
TO THE EDITOR:
The review on acquired C1 esterase inhibitor deficiency (1) reports that C2 kinin is the cause of the swelling. This peptide was originally described as a plasmin-cleavage product of C2b, and a synthetic peptide appears to possess such activity (2). However, it has not been possible to activate human plasma that is deficient in C1 inhibitor to yield a kinin other than bradykinin (3, 4), and bradykinin levels are elevated in plasma of patients with hereditary angioedema during attacks of swelling. Furthermore, swelling has never occurred in members of one unique family with a dysfunctional C1 inhibitor protein that is defective in inhibiting complement but is normal in inactivating plasma kallikrein and factor XIIa (5). These data all suggest that bradykinin is the key mediator of swelling in C1 inhibitor deficiency (whether hereditary or acquired) and that C1 inhibitor has a role as an inhibitor of bradykinen formation (Figure). Although inhibitors of plasmin or plasminogen activation, such as e-amino caproic acid, provide effective treatment of C1 inhibitor deficiency, the mechanism by which this occurs and the role of plasmin in the process are not yet clear.
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