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
-
Renin-Angiotensin System in Diabetes
Protein & Peptide Letters Unusual Food Allergy: AlioideaAllergic Reactions Overview
Recent Patents on Inflammation & Allergy Drug Discovery The Role of Anaphylatoxins C3a and C5a in Regulating Innate and Adaptive Immune Responses
Inflammation & Allergy - Drug Targets (Discontinued) Methylene Blue and Vasoplegia: Who, When, and How?
Mini-Reviews in Medicinal Chemistry Hymenoptera Venom Proteins and Peptides for Diagnosis and Treatment of Venom Allergic Patients
Inflammation & Allergy - Drug Targets (Discontinued) Omalizumab: Not Only For Asthma
Recent Patents on Inflammation & Allergy Drug Discovery Cow’s Milk Allergy: Where have we Come from and where are we Going?
Endocrine, Metabolic & Immune Disorders - Drug Targets Effects of Amine Oxidases in Allergic and Histamine-Mediated Conditions
Recent Patents on Inflammation & Allergy Drug Discovery Fixed Drug Eruption Due to Selective Hypersensitivity to Naproxen with Tolerance to other Propionic Acid NSAIDs
Recent Patents on Inflammation & Allergy Drug Discovery Cross-Reactivity in Cell-Mediated and IgE-Mediated Hypersensitivity to Glucocorticoids
Current Pharmaceutical Design Biologic Agents in the Treatment of Rheumatoid Arthritis
Current Pharmaceutical Biotechnology The Treatment of Painful Diabetic Neuropathy
Current Diabetes Reviews Extracellular Proteases as Targets for Drug Development
Current Protein & Peptide Science Hypertension in Older Patients
Current Hypertension Reviews Renin-angiotensin System Blockade at the Level of the Angiotensin Converting Enzyme or the Angiotensin Type-1 Receptor: Similarities and Differences
Current Topics in Medicinal Chemistry Managing Clopidogrel Hypersensitivity without Interrupting Therapy: The Toronto Approach
Current Vascular Pharmacology Prevention of Macrovascular Disease in Type 2 Diabetic Patients: Blockade of the Renin-Angiotensin-Aldosterone System
Current Diabetes Reviews Antineoplastic Activities, Apoptotic Mechanism of Action and Structural Properties of a Novel Silver(I) Chelate
Current Medicinal Chemistry Immediate and Late Adverse Reactions to Iodinated Contrast Media: A Pharmacological Point of View
Anti-Inflammatory & Anti-Allergy Agents in Medicinal Chemistry Kinin Receptors in Vascular Biology and Pathology
Current Vascular Pharmacology