M. Cicardi and A. Zanichelli Pages 354 - 360 ( 7 )
Acquired deficiency of C1 inhibitor (C1-INH) with angioedema symptoms (acquired angioedema, AAE) is characterized by local increase in vascular permeability (agioedema) of the skin and the gastrointestinal and oro-pharyngo-laryngeal mucosa. The mediator of symptoms is bradykinin, a potent vasoactive peptide, released from high molecular weight kininogen when it is cleaved by plasma kallikrein a serine protease controlled by C1-INH. Autoantibodies inactivating C1-INH are detected in the majority of patients and account for the deficiency. Irrespectively to the presence of anti-C1-INH autoantibodies lymphoproliferative diseases, ranging from benign monoclonal gammopathies to malignant lymphoma, are frequently associated with AAE. Demonstration that monoclonal components correspond to anti-C1-INH autoantibodies and correlation between course of lymphoma and course of AAE provide strong support to consider the two diseases expression of the same pathologic process.
Angioedema, C1 inhibitor, lymphoma, autoimmunity, vascular permeability, bradykinin, contact system
Dipartimento di Scienze Cliniche L. Sacco, Universita di Milano, Ospedale Luigi Sacco, Via G.B. Grassi 74, 20157 Milano, Italy.