Abstract
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.
Keywords: Angioedema, C1 inhibitor, lymphoma, autoimmunity, vascular permeability, bradykinin, contact system
Current Molecular Medicine
Title: The Acquired Deficiency of C1-Inhibitor: Lymphoproliferation and Angioedema
Volume: 10 Issue: 4
Author(s): M. Cicardi and A. Zanichelli
Affiliation:
Keywords: Angioedema, C1 inhibitor, lymphoma, autoimmunity, vascular permeability, bradykinin, contact system
Abstract: 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.
Export Options
About this article
Cite this article as:
Cicardi M. and Zanichelli A., The Acquired Deficiency of C1-Inhibitor: Lymphoproliferation and Angioedema, Current Molecular Medicine 2010; 10 (4) . https://dx.doi.org/10.2174/156652410791317066
DOI https://dx.doi.org/10.2174/156652410791317066 |
Print ISSN 1566-5240 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-5666 |
- Author Guidelines
- Graphical Abstracts
- Fabricating and Stating False Information
- Research Misconduct
- Post Publication Discussions and Corrections
- Publishing Ethics and Rectitude
- Increase Visibility of Your Article
- Archiving Policies
- Peer Review Workflow
- Order Your Article Before Print
- Promote Your Article
- Manuscript Transfer Facility
- Editorial Policies
- Allegations from Whistleblowers
Related Articles
-
Antibodies Against Complement System in SLE and their Potential Diagnostic Utility
Current Rheumatology Reviews Trends in the Exploration of Anticancer Targets and Strategies in Enhancing the Efficacy of Drug Targeting
Current Molecular Pharmacology Old Versus New Anticoagulants: Focus on Pharmacology
Recent Patents on Cardiovascular Drug Discovery Reporting Rates of Yellow Fever Vaccine 17D or 17DD-Associated Serious Adverse Events in Pharmacovigilance Data Bases: Systematic Review
Current Drug Safety Clopidogrel Desensitization: Background and Recommendations for Use of a Rapid (4 Hour) Protocol
Current Vascular Pharmacology Vasopeptidase Inhibition for Blood Pressure Control: Emerging Experience
Current Pharmaceutical Design Complement Activation-Related Pseudoallergy Caused by Amphiphilic Drug Carriers: The Role of Lipoproteins
Current Drug Delivery Review of Newer Anticoagulants and Anti-platelet Agents in Acute Coronary Syndrome and Cardiovascular Diseases
Cardiovascular & Hematological Agents in Medicinal Chemistry What Does a Pediatrician Need to Know About Chronic Diarrhea?
Current Pediatric Reviews Management of Food-Induced Anaphylaxis: Unsolved Challenges
Current Clinical Pharmacology Peptide and Non-peptide Antagonists Targeting Endothelin Receptors in Physiology and Pathology
Current Pharmaceutical Design Analgesia in PACU: Nonsteroidal Anti-Inflammatory Drugs
Current Drug Targets Drug Development and the Importance of Ethnicity: Lessons from Heart Failure Management and Implications for Hypertension
Current Pharmaceutical Design Prediction by Pharmacogenetics of Safety and Efficacy of Non-Steroidal Anti- Inflammatory Drugs: A Review
Current Drug Metabolism Insulin Therapy for Diabetes Epidemic: A Patent Review
Current Drug Delivery Treatment of Hereditary Angioedema: Current Perspectives
Recent Patents on Inflammation & Allergy Drug Discovery Isolated Perioperative Hypertension: Clinical Implications & Contemporary Treatment Strategies
Current Hypertension Reviews C1-Inhibitor: Structure, Functional Diversity and Therapeutic Development
Current Medicinal Chemistry Current Inhibition Concepts of Zinc Metallopeptidases Involved in Blood Pressure Regulation
Current Enzyme Inhibition Serine Proteinase Inhibitors in the Skin: Role in Homeostasis and Disease
Current Protein & Peptide Science