Vasculitis in Clinical Practice (eBook)

Richard A. Watts, DM MA FRCP is a Senior Lecturer at the University of East Anglia School of Medicine, Health and Policy Practice and a Consultant Rheumatologist at Ipswich Hospital NHS Trust, in the UK.David G.I. Scott, MD FRCP is a Consultant Rheumatologist at Norfolk and Norwich University Hospital NHS Trust, and an Honorary Professor at the University of East Anglia, School of Medicine, Health Policy and Practice, in the UK.

Vasculitis in Clinical Practice 2
Preface 4
Contents 5
Contributors 16
Abbreviations 17
Part 1: Introduction 19
Chapter 1: Definitions 20
1.1 General Introduction 20
1.2 Definitions 21
Chapter 2: Classification and Epidemiology 24
2.1 Introduction 24
2.2 ACR (1990) Criteria 25
2.3 Chapel Hill Consensus Definitions 26
2.4 Epidemiology 26
2.5 Etiological Factors 27
Key Points 28
References 28
Chapter 3: General Presentation of the Vasculitides 29
3.1 Introduction 29
3.2 Pattern Recognition 29
3.3 Laboratory Investigations 32
3.4 Differential Diagnosis 34
Key Points 34
References 35
Chapter 4: General Principles of Treatment 36
4.1 Treatment 36
4.2 Remission Induction 36
4.2.1 Large Vessel Disease 37
4.2.2 Medium Vessel Disease 37
4.2.3 Medium/Small Vessel Vasculitis 37
4.2.4 Small Vessel Vasculitis 39
4.3 Maintenance Therapy 40
4.4 Long-Term Follow-Up 40
4.5 Relapsing Disease 40
4.6 Refractory Disease 41
4.7 Specific Drugs Used to Treat Vasculitis 42
4.7.1 Cyclophosphamide 42
4.7.2 Glucocorticoids 43
4.7.3 Methotrexate 43
4.7.4 Plasma Exchange 44
4.8 Monitoring 44
4.9 Patient Advice 45
4.10 Detection and Prevention of the Adverse Effects of Therapy 45
4.10.1 Osteoporosis 45
4.10.2 Vaccinations 46
4.10.3 Pneumocystis Jirovecii Infection 46
4.10.4 Cyclophosphamide-Induced Bladder Toxicity 46
Key Points 46
References 47
Part 2: Features of Individual Diseases 48
Chapter 5: Giant Cell Arteritis 49
5.1 Introduction 49
5.2 Definition and Classification 49
5.3 Epidemiology 50
5.4 Etiology 50
5.5 Clinical Features 51
5.5.1 Systemic 51
5.5.2 Craniofacial 51
5.5.3 Ophthalmic 53
5.5.4 Neurologic 53
5.5.5 Extracranial Artery 53
5.6 Laboratory Features 54
5.6.1 immunology 54
5.6.2 Imaging 54
5.6.3 Pathology 55
5.7 Diagnosis 56
5.8 Assessment of Disease Activity 57
5.9 Treatment 57
5.10 Prognosis 58
Key Points 58
References 59
Chapter 6: Takayasu Arteritis 60
6.1 Introduction 60
6.2 Definition and Classification 60
6.3 Epidemiology 61
6.4 Etiology 61
6.5 Clinical Features 62
6.5.1 Systemic 62
6.5.2 Vascular 62
6.6 Laboratory Features 62
6.6.1 Immunology 63
6.6.2 Imaging 63
6.6.3 Pathology 65
6.7 Diagnosis 66
6.8 Assessment of Disease Activity 66
6.9 Treatment 66
6.9.1 Pharmacological Therapy 66
6.9.2 Surgery 67
6.10 Prognosis 67
Key Points 68
References 68
Chapter 7: Wegener’s Granulomatosis 69
7.1 Introduction 69
7.2 Definition and Classification 69
7.3 Epidemiology 70
7.4 Etiology 70
7.5 Clinical Features 71
7.5.1 Systemic 71
7.5.2 Pulmonary 71
7.5.3 Cutaneous 71
7.5.4 ENT 72
7.5.5 Gastrointestinal 73
7.5.6 Neurological 73
7.5.7 Renal 73
7.5.8 Eye 73
7.5.9 Musculoskeletal 74
7.5.10 Cardiac 74
7.6 Laboratory Features 74
7.6.1 Immunology 75
7.6.2 Imaging 75
7.6.3 Pathology 77
7.7 Diagnosis 78
7.7.1 Differential Diagnosis 78
7.7.2 Assessment of Organ Involvement 78
7.8 Assessment of Disease Activity 79
7.9 Treatment 79
7.9.1 Remission Induction 80
7.9.2 Maintenance 80
7.10 Prognosis 80
Key Points 81
References 82
Chapter 8: Churg–Strauss Syndrome 83
8.1 Introduction 83
8.2 Definition and Classification 83
8.3 Epidemiology 84
8.4 Etiology 85
8.5 Clinical Manifestations 85
8.5.1 Pulmonary 85
8.5.2 Cutaneous 86
8.5.3 Gastrointestinal 87
8.5.4 Neurological 87
8.5.5 Cardiac 88
8.5.6 Renal 88
8.5.7 ENT 88
8.5.8 Ophthalmic 88
8.5.9 Musculoskeletal 89
8.6 Laboratory Features 89
8.6.1 Immunology 90
8.6.2 Imaging 90
8.6.3 Pathology 90
8.7 Diagnosis 90
8.7.1 Differential Diagnosis 90
8.7.2 Assessment of Organ Involvement 91
8.8 Assessment of Disease Activity 91
8.9 Treatment 92
8.9.1 Remission Induction 92
8.9.2 Maintenance 92
8.10 Prognosis 93
Key Points 93
References 93
Chapter 9: Microscopic Polyangiitis 95
9.1 Introduction 95
9.2 Definition and Classification 95
9.3 Epidemiology 95
9.4 Etiology 96
9.5 Clinical Features 96
9.5.1 Systemic 96
9.5.2 Renal 96
9.5.3 Pulmonary 96
9.5.4 Cutaneous 98
9.5.5 Neurological 98
9.5.6 Cardiac 98
9.5.7 Gastrointestinal 98
9.5.8 Otorhinolaryngeal 98
9.5.9 Ocular 99
9.5.10 Venous Thromboembolism 99
9.6 Laboratory Features 99
9.6.1 immunology 99
9.6.2 Pathology 99
9.7 Diagnosis 100
9.7.1 Differential Diagnosis 100
9.7.2 Assessment of Organ Involvement 101
9.8 Assessment of Disease Activity 101
9.9 Treatment 102
9.9.1 Remission Induction 102
9.9.2 Maintenance 103
9.10 Prognosis 103
Key Points 104
References 104
Chapter 10: Polyarteritis Nodosa 106
10.1 Introduction 106
10.2 Definition and Classification 106
10.3 Epidemiology 106
10.4 Etiology 108
10.5 Clinical Features 108
10.5.1 Systemic 108
10.5.2 Cutaneous 108
10.5.3 Musculoskeletal 108
10.5.4 Neurological 109
10.5.5 Renal 110
10.5.6 Gastrointestinal 110
10.5.7 Cardiac 110
10.5.8 Orchitis 111
10.5.9 Other 111
10.6 Laboratory Features 111
10.6.1 Immunology 112
10.6.2 Viral Serology 112
10.6.3 Imaging 112
10.6.4 Pathology 113
10.7 Diagnosis 114
10.7.1 Differential Diagnosis 114
10.7.2 Assessment of Organ Involvement 114
10.8 Assessment of Disease Activity 115
10.9 Treatment 115
10.9.1 Non-HBV-PAN 115
10.9.2 Virus-Associated PAN 115
10.10 Prognosis 116
Key Points 117
References 117
Chapter 11: Kawasaki Disease 118
11.1 Introduction 118
11.2 Definition and Classification 118
11.3 Epidemiology 119
11.4 Etiology 119
11.5 Clinical Features 119
11.5.1 Mucosal 120
11.5.2 Cutaneous 120
11.5.3 Ophthalmic 121
11.5.4 Cardiovascular 121
11.6 Laboratory Features 122
11.6.1 Cardiac Investigations 122
11.6.2 Immunology 122
11.6.3 Pathology 122
11.7 Diagnosis 123
11.8 Assessment of Disease Activity 123
11.9 Treatment 123
11.10 Prognosis 124
Key Points 125
References 125
Chapter 12: Henoch–Schönlein Purpura 126
12.1 Introduction 126
12.2 Definition and Classification 126
12.3 Epidemiology 127
12.4 Etiology 128
12.5 Clinical Features 128
12.5.1 Cutaneous 128
12.5.2 Gastrointestinal 128
12.5.3 Musculoskeletal 130
12.5.4 Renal 130
12.6 Laboratory Features 130
12.6.1 Renal Function 131
12.6.2 Immunology 131
12.6.3 Pathology 131
12.7 Diagnosis 132
12.8 Assessment of Disease Activity 132
12.9 Treatment 132
12.10 Prognosis 133
Key Points 133
References 134
Chapter 13: Behçet’s Disease 135
13.1 Introduction 135
13.2 Definition and Classification 135
13.3 Epidemiology 135
13.4 Etiology 136
13.5 Clinical Features 136
13.5.1 Orogenital 136
13.5.2 Cutaneous 137
13.5.3 Ophthalmic 138
13.5.4 Neurological 139
13.5.5 Vascular 140
13.5.6 Musculoskeletal 140
13.5.7 Gastrointestinal 140
13.5.8 Renal 140
13.6 Laboratory Features 141
13.6.1 Immunology 141
13.6.2 Genetic 141
13.6.3 Synovial Fluid Examination 141
13.6.4 Cerebrospinal Fluid 141
13.6.5 Imaging 141
13.6.6 Pathology 142
13.7 Diagnosis 142
13.8 Assessment of Disease Activity 142
13.9 Treatment 143
13.9.1 Mucocutaneous 143
13.9.2 Ocular 143
13.9.3 Vasculopathy 144
13.9.4 Gastrointestinal 144
13.9.5 Musculoskeletal 144
13.9.6 Neurological 144
13.10 Prognosis 145
Key Points 146
References 146
Chapter 14: Cryoglobulinemic Vasculitis 147
14.1 Introduction 147
14.2 Definition 147
14.3 Epidemiology 147
14.4 Etiology 148
14.5 Clinical Features 148
14.5.1 Systemic 148
14.5.2 Cutaneous 148
14.5.3 Neurological 148
14.5.4 Renal 149
14.5.5 Musculoskeletal 149
14.6 Laboratory Features 149
14.6.1 Viral Serology 150
14.6.2 Immunology 150
14.6.3 Pathology 150
14.7 Diagnosis 150
14.8 Assessment of Disease Activity 151
14.9 Treatment 151
14.10 Prognosis 151
Key Points 152
References 152
Chapter 15: Vasculitis Mimics 153
15.1 Introduction 153
15.2 Cholesterol Crystal Embolism 153
15.2.1 Clinical Features 154
15.2.2 Laboratory Features 156
15.2.3 Treatment 157
15.3 Calciphylaxis 157
15.4 Cardiac Myxoma 157
15.4.1 Clinical Features 157
15.4.2 Treatment 158
15.5 Infective Endocarditis 158
15.6 Fibromuscular Dysplasia 158
15.7 Chronic Ergotism 159
15.8 Köhlmeier–Degos Disease 159
15.9 Cryofibrinogenemia 160
15.10 Radiation Vasculopathy 161
15.11 Cocaine Abuse 161
15.12 Scurvy 161
15.13 Sweet’s Syndrome 163
Key Points 163
References 164
Chapter 16: Secondary Vasculitis 165
16.1 Introduction 165
16.2 Definition 165
16.3 Epidemiology 165
16.4 Etiology 166
16.5 Infection and Vasculitis 166
16.6 Vasculitis and Malignancy 168
16.7 Drug-Induced Vasculitis 170
16.8 Systemic Rheumatoid Vasculitis 171
16.9 Systemic Lupus Erythematosus 173
16.10 Sjögren’s Syndrome 174
16.11 Spondyloarthopathies 174
Key points 175
References 175
Chapter 17: Primary Angiitis of the Central Nervous System Vasculitis 177
17.1 Introduction 177
17.2 Definition and Classification 177
17.3 Epidemiology 178
17.4 Etiology 178
17.5 Clinical Features 178
17.5.1 Systemic 178
17.5.2 Neurological 178
17.6 Laboratory Features 179
17.6.1 Cerebrospinal Fluid 179
17.6.2 Imaging 179
17.6.3 Pathology 180
17.6.4 Electroencephalography 180
17.7 Diagnosis 180
17.8 Assessment of Disease Activity 180
17.9 Treatment 180
17.10 Prognosis 181
Key Points 181
References 182
Chapter 18: Relapsing Polychondritis 183
18.1 Introduction 183
18.2 Definition and Classification 183
18.3 Epidemiology 183
18.4 Etiology 183
18.5 Clinical Features 184
18.5.1 Systemic 184
18.5.2 Cartilage Inflammation 184
18.5.3 Pulmonary 185
18.5.4 Ophthalmic 186
18.5.5 Musculoskeletal 187
18.5.6 Cutaneous 187
18.5.7 Cardiovascular 187
18.6 Laboratory Features 187
18.6.1 Immunology 187
18.6.2 Imaging 187
18.6.3 Pathology 188
18.7 Diagnosis 188
18.8 Assessment Disease Activity 188
18.9 Treatment 188
18.10 Prognosis 189
Key Points 189
References 189
Chapter 19: Cogan’s Syndrome 190
19.1 Introduction 190
19.2 Definition and Classification 190
19.3 Epidemiology 190
19.4 Etiology 190
19.5 Clinical Features 191
19.5.1 Systemic 191
19.5.2 Ocular 191
19.5.3 Vestibular and Auditory 191
19.5.4 Vascular 192
19.5.5 Neurological 192
19.6 Laboratory Features 192
19.6.1 Immunology 192
19.6.2 Pathology 192
19.7 Diagnosis 193
19.8 Assessment of Disease Activity 193
19.9 Treatment 193
19.10 Prognosis 193
Key Points 194
References 194
Index 195