To the Editor.—Two types of acquired angioedema (AAE) are recognized today; type I with low levels of functional C1-inhibitor (C1-INH), no detectable autoantibody to C1-INH, and an underlying disease, such as a B-cell disorder,1 and type II with moderately depressed functional C1-INH levels, anti–C1-INH autoantibodies in circulation without detectable underlying disease.2-4 We describe the long-term follow-up of a patient with AAE type II.
Report of a Case.—A woman, born in 1921, first presented with an attack of angioedema in December 1980. Based on complement studies and patient history, an AAE was diagnosed in December 1981. Therapy with danazol and later with tranexamic acid was initiated. Recurrent life-threatening attacks with need for the infusion of fresh frozen plasma indicated ineffectiveness of these therapies. Several investigations failed to detect a malignant neoplasia or a dysproteinemia. However, in August 1983, a giant subserous myoma of the fundus of the
SPÄTH PJ, WÜTHRICH B, MATTER L, LOOS M, ALSENZ J. Acquired Angioedema and Anti–C1-Inhibitor Autoantibody. Arch Intern Med. 1989;149(5):1213–1216. doi:10.1001/archinte.1989.00390050159036
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