Background: Oral contraceptives can precipitate attacks of hereditary angioedema (ANE) or induce acquired forms. Objective: We studied 5 patients who had an ANE which had begun under oral contraception and disappeared after stopping the pill. Methods: We explored the clinical and biological characteristics of these patients. Results: The symptoms developed during the first year or later after starting contraception; the patients reported relapsing swelling of the lips, hands, larynx and abdomen. All women had normal serum C4 and C1 inhibitor (C1Inh) antigen levels, but a lowered C1Inh activity, with a marked protein cleavage on the immunoblot. The suppression of the pill was associated with the regression of the edema and normalization of C1Inh function. Conclusion: The mechanism of these ANE is unknown. The could be due to a modulation of C1Inh expression upon androgens or an imbalance between coagulation proteins favoring C1Inh cleavage by its target proteases.

Keywords:
Angioedema, Oral contraception, C1Inh
1.
Bork K, Barnstedt SE, Koch P, Traupe H: Hereditary angioedema with normal C1 inhibitor activity in women. Lancet 2000;356:213–217.
2.
McGlinchey PG, McCluskey DR: Hereditary angioedema precipitated by estrogens replacement therapy in a menopausal woman. Am J Med Sci 2000;320:212–213.
3.
Warin RP, Cunliffe WJ, Greaves MW, Wallington TB: Recurrent angioedema: Familial and estrogens induced. Br J Dermatol 1986;115:731–734.
4.
Drouet C, Alibeu C, Ponard D, Arlaud G, Colomb M: A sensitive method to assay blood complement C1 inhibitor activity. Clin Chim Acta 1988;174:121–130.
5.
Binkley KE, Davis A: Clinical, biochemical, and genetic characterization of a novel estrogen dependent inherited form of angioedema. J Allergy Clin Immunol 2000;106:546–550.
6.
Gordon EM, Ratnoff OD, Saito H, Donaldson VH, Pensky J, Joes PK: Rapid fibrinolysis, augmented Hageman factor (factor XII) titers, and decreased C1 esterase inhibitor titers in women taking oral contraceptives. J Lab Clin Med 1980;96:762–769.
7.
Pichler WJ, Lehner R, Spath PJ: Recurrent angioedema associated with hypogonadism or anti-androgen therapy. Ann Allergy 1989;63:301–305.
8.
Fields T, Ghebrehiwet B, Kaplan AP: Kinin formation in HANE plasma: Evidence against kinin derivation from C2 and in support of spontaneous formation of bradykinin. J Allergy Clin Immunol 1983;72:54–60.
9.
Nussberger J, Cugno M, Amstutz C, Cicardi M, Pellacani A, Agostoni A: Plasma bradykinin in angio-oedema. Lancet 1998;351:1693–1697.
10.
Czendlik C, Lämmle B, Duckert F: Cold promoted activation and factor XII, prekallikrein and C1-inhibitor. Thromb Haemost 1985;53:242–244.
11.
Van Royen EA, Lohman S, Voss M, Pondman KW: C1 inactivator and cold promoted activation of factor VII. J Lab Clin Med 1978;92:152–163.
12.
Nilsson T, Back O: On the role of C1 esterase inhibitor in cold urticaria. Acta Derm Venereol 1984;64:197–202.
© 2003 S. Karger AG, Basel
2003
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.