The acquired deficiency of C1-inhibitor: lymphoproliferation and angioedema.

Abstract

Acquired deficiency of C1 inhibitor (C1-INH) with angioedema symptoms (acquired angioedema, AAE) is characterized by local increase in vascular permeability (angioedema) 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.

• Authors: M Cicardi, A Zanichelli
• Journal: Current molecular medicine (Curr Mol Med) Vol. 10 Issue 4 Pg. 354-60 (Jun 2010) ISSN: 1875-5666 [Electronic] Netherlands
• PMID: 20455857 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Review)

Chemical References

• Autoantibodies
• Complement C1 Inhibitor Protein
• Bradykinin

Topics

• Angioedema (blood, etiology, immunology)
• Autoantibodies (blood)
• B-Lymphocytes (immunology)
• Bradykinin (physiology)
• Capillary Permeability
• Complement C1 Inhibitor Protein (immunology, metabolism)
• Female
• Humans
• Lymphoproliferative Disorders (blood, etiology, immunology)
• Male
• Models, Biological