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Angioedema, An Issue of Immunology and Allergy Clinics of North America, E-Book

Angioedema, An Issue of Immunology and Allergy Clinics of North America, E-Book

Marc Riedl

(2017)

Additional Information

Book Details

Abstract

This issue of Immunology and Allergy Clinics, guest edited by Mariana Castells, is devoted to Drug Hypersensitivity and Desensitizations. Articles in this issue include: Principles and Practice of Drug Desensitization; Incidence and Prevalence of Drug Hypersensitivity; Drug-Induced Anaphylaxis; Penicillin and Beta Lactam Hypersensitivity; Platins Chemotherapy Hypersensitivity: Prevalence and Management; Monoclonal Antibodies Hypersensitivity: Prevalence and Management; Injectable Drugs and MoAbs Reactions and Management with Desensitization; Delayed Cutaneous Reactions to Antibiotics, Management with Desensitization; Management of Children with Hypersensitivity to Antibiotics and MoAbs; Taxenes Hypersensitivity and Management; Progesterone Autoimmune Dermatitis; Severe Delayed Drug Reaction: Role of Genetics and Viral Infections; andAspirin and NSAIDS Hypersensitivity and Management.

Table of Contents

Section Title Page Action Price
Front Cover Cover
Angioedema i
Copyright\r ii
Contributors iii
CONSULTING EDITOR iii
EDITOR iii
AUTHORS iii
Contents vii
Foreword: Angioedema: An Orphan Symptom with Its Own Orphan Disease vii
Preface: Angioedema: Challenges and Insights vii
The Clinical Evaluation of Angioedema vii
Histaminergic Angioedema vii
Angiotensin-converting Enzyme Inhibitor and Other Drug-associated Angioedema vii
Acquired C1 Inhibitor Deficiency viii
Pathogenesis of Hereditary Angioedema: The Role of the Bradykinin-Forming Cascade viii
Laboratory Approaches for Assessing Contact System Activation viii
Acute Management of Hereditary Angioedema Attacks viii
Prophylactic Therapy for Hereditary Angioedema ix
Hereditary Angioedema with Normal C1 Inhibitor: Update on Evaluation and Treatment ix
Emerging Therapies in Hereditary Angioedema ix
Burden of Illness and Quality-of-Life Measures in Angioedema Conditions ix
Pharmacoeconomics of Orphan Disease Treatment with a Focus on Hereditary Angioedema x
IMMUNOLOGYAND ALLERGY\rCLINICS OF NORTH AMERICA\r xi
FORTHCOMING ISSUES xi
November 2017 xi
February 2018 xi
May 2018 xi
RECENT ISSUES xi
May 2017 xi
February 2017 xi
November 2016 xi
Foreword: Angioedema: An Orphan Symptom with Its Own Orphan Disease\r xiii
Preface:\rAngioedema: Challenges and Insights xv
The Clinical Evaluation of Angioedema 449
Key points 449
INTRODUCTION 449
SIGNS AND SYMPTOMS OF ANGIOEDEMA 450
PATHOPHYSIOLOGY OF ANGIOEDEMA 450
Histamine-Mediated Pathways 450
Bradykinin-Mediated Pathways 452
Angioedema Resulting Through Cyclooxygenase 1 Inhibition 454
Type III Hypersensitivity Reactions 454
CLASSIFICATION OF ANGIOEDEMA 454
APPROACH TO THE CLINICAL EVALUATION AND DIAGNOSTIC WORKUP 454
Histamine-Mediated Angioedema 454
Clinical features 454
Allergic angioedema 454
Pseudoallergic angioedema 455
Idiopathic angioedema 455
DIAGNOSTIC EVALUATION OF HISTAMINE-MEDIATED ANGIOEDEMA 456
Bradykinin-Mediated Angioedema 457
Clinical features 457
Hereditary angioedema 457
Acquired agioedema 458
Angiotensin-converting enzyme–inhibitor induced angioedema 459
DIAGNOSTIC EVALUATION OF BRADYKININ-MEDIATED ANGIOEDEMA 459
Essential Components of the Clinical History 460
An Algorithmic Approach to Diagnosis of Angioedema 460
FUTURE CONSIDERATIONS 460
REFERENCES 464
Histaminergic Angioedema 467
Key points 467
INTRODUCTION 467
ACUTE ANGIOEDEMA 469
Allergic (Immunoglobulin E–Mediated) Angioedema 469
Presentation 469
Pathophysiology 469
Nonallergic Mast-Cell–Mediated Acute Angioedema 470
Presentation 470
Pathophysiology 470
CHRONIC ANGIOEDEMA 470
Histamine-Mediated Angioedema with Chronic Urticaria 470
Physical Urticaria 471
Presentation 471
Angiotensin-converting Enzyme Inhibitor and Other Drug-associated Angioedema 483
Key points 483
INTRODUCTION 483
ANGIOEDEMA ASSOCIATED WITH NONSTEROIDAL ANTIINFLAMMATORY DRUG USE 484
ANGIOTENSIN-CONVERTING ENZYME INHIBITOR–ASSOCIATED ANGIOEDEMA 485
Clinical Features of Angiotensin-converting Enzyme Inhibitor–associated Angioedema 485
Epidemiology of and Risk Factors for Angiotensin-converting Enzyme Inhibitor–associated Angioedema 485
Pathophysiology of Angiotensin-converting Enzyme Inhibitor–associated Angioedema 486
Genetics of Angiotensin-converting Enzyme Inhibitor–associated Angioedema 487
Management of Angiotensin-converting Enzyme Inhibitor–associated Angioedema 488
DIPEPTIDYL PEPTIDASE 4 INHIBITORS AND ANGIOEDEMA 489
COMBINED ANGIOTENSIN RECEPTOR BLOCKER/NEPRILYSIN INHIBITOR AND ANGIOEDEMA 489
TISSUE-TYPE PLASMINOGEN ACTIVATOR–INDUCED ANGIOEDEMA 490
SUMMARY 490
REFERENCES 490
Acquired C1 Inhibitor Deficiency 497
Key points 497
INTRODUCTION 497
EPIDEMIOLOGY 498
RISK FACTORS 499
CLINICAL FEATURES 499
PATHOPHYSIOLOGY 500
DIFFERENTIAL DIAGNOSIS 501
MANAGEMENT 502
Symptom Control 502
Acute Treatment 502
Prophylaxis 503
Short-term Prophylaxis 503
Long-term Prophylaxis 503
Treatment of Acquired Angioedema due to C1-INH Deficiency and Underlying Systemic Conditions 504
PROGNOSIS 504
CURRENT CONTROVERSIES 504
Pathophysiology 504
C1 Esterase Inhibitor Replacement Therapy 504
Rituximab Therapy 507
REFERENCES 507
Pathogenesis of Hereditary Angioedema 513
Key points 513
INTRODUCTION 513
THE ROLE OF C1 INHIBITOR IN COMPLEMENT ACTIVATION 514
BRADYKININ IS THE MEDIATOR OF SWELLING IN HEREDITARY ANGIOEDEMA TYPES I AND II 515
THE PATHWAY(S) OF BRADYKININ FORMATION IN TYPES I AND II HEREDITARY ANGIOEDEMA 517
VASOPERMEABILITY OWING TO BRADYKININ 518
ASSEMBLY AND ACTIVATION OF THE BRADYKININ-FORMING CASCADE ON ENDOTHELIAL CELLS 518
HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR: NEW FORMS OF HEREDITARY ANGIOEDEMA 521
REFERENCES 522
Laboratory Approaches for Assessing Contact System Activation 527
Key points 527
INTRODUCTION 527
BRADYKININ-MEDIATED ANGIOEDEMA 528
Role of Bradykinin in Hereditary Angioedema due to C1 Inhibitor Deficiency 528
Role of Bradykinin in Hereditary Angioedema with Normal C1 Inhibitor 529
Role of Bradykinin in Other Forms of Angioedema 529
LABORATORY DIAGNOSIS OF ANGIOEDEMA 530
Angioedema Associated with C1 Inhibitor Deficiency 530
Bradykinin-Mediated Angioedema with Normal C1 Inhibitor 531
BIOMARKERS OF CONTACT SYSTEM ACTIVATION 531
Measurement of C1 Inhibitor Levels 531
C4 Levels 532
Measurement of Protease-Inhibitor Complexes 532
Cleavage of Zymogen Proteases 533
High-Molecular-Weight Kininogen Cleavage 533
Bradykinin Levels 533
Measurement of Kallikrein Activity 533
Other Assays 533
SUMMARY 533
REFERENCES 535
Acute Management of Hereditary Angioedema Attacks 541
Key points 541
INTRODUCTION 541
MEDICATIONS FOR TREATING ACUTE ATTACKS 542
PLASMA-DERIVED C1 INHIBITOR THERAPY 542
Berinert 542
Studies of efficacy 542
Cinryze 542
Onset of Action of Plasma-Derived C1 Inhibitors 543
Rebound and Relapse 543
SPECIFIC CIRCUMSTANCES 543
Laryngeal Attacks 543
Treatment in Pediatric Patients 543
Pregnancy and Breast Feeding 544
ADVERSE EVENTS WITH C1 INHIBITOR PRODUCTS 544
Viral Transmission 544
Thromboembolic Events 544
Immunogenicity 545
INDICATION AND ADMINISTRATION 545
RECOMBINANT C1 INHIBITOR CONESTAT ALFA 545
Introduction 545
Trial Data 547
Onset of Action 547
Rebound and Relapse 547
Response by Anatomic Location 547
SPECIAL POPULATIONS 548
Pediatric Use 548
Pregnancy and Breast Feeding 548
Adverse Events 548
Immunogenicity 548
Thrombogenicity 548
Administration 548
ECALLANTIDE 548
Introduction 548
Clinical Trial Data 549
Onset of Action 549
Rebound 549
Laryngeal Attacks 549
Special Populations 549
Adverse Events 550
Anaphylaxis 550
Immunogenicity 550
Administration 550
ICATIBANT 550
Introduction 550
Studies of Efficacy 550
Laryngeal Attacks 551
Adverse Events 551
Indications and Administration 551
Self-Administration 551
Other Considerations 552
Lack of efficacy of tranexamic acid for acute hereditary angioedema attacks 552
FRESH FROZEN PLASMA 552
Preparedness to Intubate 552
Patient Self-Administration 552
SUMMARY 552
REFERENCES 553
Prophylactic Therapy for Hereditary Angioedema 557
Key points 557
INTRODUCTION 557
WHEN PROPHYLAXIS IS INDICATED 558
ATTENUATED ANDROGENS 558
Efficacy 558
Side Effects of Attenuated Androgens 559
Abnormalities in Hepatic Enzymes 559
Liver Neoplasms 560
Cardiovascular Risks and Lipid Profiles 560
Hemorrhagic Cystitis 561
Current Guidelines for the Use of Androgen Therapy as Prophylaxis 561
C1-INHIBITOR 561
Efficacy 561
Dosing and Delivery Route 562
Ongoing Developments 562
Side Effects 562
Thromboembolic Events 563
Theoretic Viral/Prion Risk 563
TRANEXAMIC ACID 563
Safety Profile and Side Effects 563
PROGESTAGENS 564
Side Effect Profile 564
Use as Prophylaxis 564
SHORT-TERM PROPHYLAXIS 564
Introduction 564
Options for Short-term Prophylaxis 565
C1 Inhibitor 565
Attenuated Androgens 565
Tranexamic Acid 565
SUMMARY 566
REFERENCES 566
Hereditary Angioedema with Normal C1 Inhibitor 571
Key points 571
INTRODUCTION 571
THE PATHOPHYSIOLOGY OF HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR 572
THE GENETICS OF HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR 573
CLINICAL FEATURES OF HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR 575
DIAGNOSIS AND DIFFERENTIAL DIAGNOSIS OF HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR 576
Diagnosis 576
Differential Diagnosis 576
TREATMENT OF HEREDITARY ANGIOEDEMA WITH NORMAL C1 INHIBITOR 577
On-demand Treatment 577
Short-term Prophylaxis 578
Long-term Prophylaxis 578
REFERENCES 579
Emerging Therapies in Hereditary Angioedema 585
Key points 585
INTRODUCTION 585
HISTAMINERGIC VERSUS BRADYKININ PATHWAYS 586
Treatment of Histamine Versus Bradykinin-Mediated Angioedema 587
RECENT DEVELOPMENTS IN HEREDITARY ANGIOEDEMA THERAPY 587
Hereditary Angioedema Therapy for Pediatrics 587
Clinical trials 587
EMERGING THERAPIES FOR LONG-TERM PROPHYLAXIS 588
Treatments in Human Studies 588
Complement component 1 esterase inhibitor concentrates 588
Subcutaneous complement component 1 esterase inhibitor 588
Recombinant human C1INH 590
Monoclonal antibody inhibitor of kallikrein 591
Lanadelumab (DX2930 or SHP643) 591
Oral kallikrein inhibitors 591
Avoralstat (BCX4161) 591
BCX7353 591
KVD818 592
Antisense targeting prekallikrein 592
Ionis PKKRx 592
Treatments in Preclinical Studies 592
Factor XII 592
ALN-F12 592
ARC-F12 592
Monoclonal antibody antifactor XIIa 593
Gene therapy 593
ACUTE THERAPY: CONCEPTUAL 593
Oral Kallikrein Inhibitor 593
FUTURE CONSIDERATIONS AND SUMMARY 593
REFERENCES 594
Burden of Illness and Quality-of-Life Measures in Angioedema Conditions 597
Key points 597
INTRODUCTION 598
HEALTH-RELATED QUALITY-OF-LIFE QUESTIONNAIRES 598
Generic Questionnaires 598
Short Form 36 Health Survey 598
EuroQoL 598
Dermatology-Specific Questionnaires 599
Dermatology Life Quality Index 599
Skindex-29 599
Angioedema-Specific Questionnaires 599
AE-QoL 599
HAE-QoL 599
Other Health-Related Quality-of-Life Measures 600
Quality-adjusted life-year 600
HEALTH-RELATED QUALITY OF LIFE AND BURDEN OF DISEASE IN HISTAMINERGIC ANGIOEDEMA 600
HEALTH-RELATED QUALITY OF LIFE AND BURDEN OF DISEASE IN HEREDITARY ANGIOEDEMA CAUSED BY C1-INHIBITOR DEFICIENCY 601
HEALTH-RELATED QUALITY OF LIFE AND BURDEN OF DISEASE IN OTHER BRADYKININ-INDUCED ANGIOEDEMAS 611
FUTURE CONSIDERATIONS/SUMMARY 611
REFERENCES 612
Pharmacoeconomics of Orphan Disease Treatment with a Focus on Hereditary Angioedema 617
Key points 617
OVERVIEW 617
ORPHAN DRUG DEVELOPMENT POLICIES AND REIMBURSEMENT ISSUES 618
PREVALENCE OF ORPHAN DISEASES 618
IMPACT OF NEW THERAPIES FOR HEREDITARY ANGIOEDEMA 619
COST OF HEREDITARY ANGIOEDEMA CARE BEFORE 2008 IN THE UNITED STATES 622
BURDEN OF DISEASE 622
INNOVATIVE TREATMENT PARADIGMS 623
SUMMARY 624
REFERENCES 625