Pediatrics in Systemic Autoimmune Diseases (eBook)

Cover 1
Contents 14
Preface 6
Series Editor 8
Volume Editors 10
List of Contributors 12
Chapter 1. Oligoarticular and Polyarticular Juvenile Idiopathic Arthritis 16
1. Introduction 16
2. Prevalence/epidemiology 17
3. Etiology and pathogenesis 17
4. Clinical manifestations 19
5. Diagnostic investigations 20
6. Differential diagnosis 22
7. Treatment 23
8. Prognosis 25
References 25
Chapter 2. The Juvenile-Onset Spondyloarthritides 30
1. Introduction 30
2. Epidemiology 32
3. Pathogenesis 32
4. Clinical manifestations 33
5. Clinical forms 36
6. Therapeutic approach 41
References 44
Chapter 3. Systemic Juvenile Idiopathic Arthritis 50
1. Introduction 50
2. Prevalence and epidemiology 50
3. Pathogenesis of S-JIA 50
4. Common clinical manifestations 52
5. Growth 54
6. Less common clinical manifestations 56
7. Macrophage activation syndrome 57
8. Laboratory investigations 59
9. Radiologic investigations 59
10. Differential diagnosis 60
11. Treatment 62
12. Disease course, outcome and prognosis 63
13. Mortality 64
References 65
Chapter 4. Macrophage Activation Syndrome 70
1. Introduction 70
2. Clinical features 70
3. Epidemiology 71
4. Nomenclature and classification 72
5. Pathogenesis 73
6. Diagnostic guidelines 74
7. Management 75
References 76
Chapter 5. Systemic Lupus Erythematosus: Etiology, Pathogenesis, Clinical Manifestations, and Management 80
1. Introduction 80
2. Definition/classification 80
3. Epidemiology 81
4. Genetics 81
5. Etiology/pathogenesis 81
6. Clinical manifestations and treatment 82
7. General aspects of management 87
8. Course and prognosis 88
9. Alternate forms of lupus: neonatal, discoid, drug-induced 88
References 89
Chapter 6. Neonatal Lupus Syndromes 92
1. Introduction 92
2. Clinical features 92
3. Epidemiology 93
4. Pathogenesis 94
5. Prognosis 96
6. Treatment 97
References 99
Chapter 7. Juvenile Dermatomyositis 104
1. Introduction 104
2. Incidence of JDM 104
3. JDM epidemiology 104
4. Pathogenesis 105
5. Clinical manifestations 105
6. Diagnostic investigations 108
7. Diagnosis 109
8. Treatment 110
Acknowledgements 112
References 112
Chapter 8. Localized Scleroderma in Children 114
1. Introduction 114
2. Epidemiology 114
3. Etiology and pathogenesis 114
4. Clinical manifestations 115
5. Diagnostic investigations 117
6. Treatment 118
References 120
Chapter 9. Juvenile Systemic Sclerosis 122
1. Introduction 122
2. Incidence and prevalence 122
3. Classification and epidemiology 122
4. Etiology/pathogenesis 123
5. Clinical manifestations 123
6. Diagnostic investigations 128
7. Differential diagnosis 129
8. Treatment 130
References 131
Chapter 10. Episodic Autoinflammatory Disorders in Children 134
1. Introduction 134
2. Familial Mediterranean Fever (FMF) 134
3. TNF-Receptor Associated Periodic Syndrome (TRAPS) 136
4. Hyper IgD Syndrome (HIDS) 138
5. Cryopyrin Associated Periodic Syndromes (CAPS) 139
6. Pyogenic sterile Arthritis, Pyoderma gangrenosum, and Acne syndrome (PAPA) 142
7. Periodic Fever with Aphthous stomatitis, Pharyngitis and Adenitis (PFAPA) 143
8. Differential diagnosis 146
Acknowledgements 147
References 147
Chapter 11. Kawasaki Disease 152
1. Introduction 152
2. Epidemiology 152
3. Etiology 153
4. Pathogenesis 154
5. Clinical manifestations 154
6. Laboratory findings 155
7. Atypical and incomplete KD 156
8. Infants and adolescents 157
9. Therapy 159
10. Cardiac follow-up 160
11. Vaccinations 161
References 162
Chapter 12. Henoch-Schönlein Purpura, Polyarteritis Nodosa, Wegener’s Granulomatosis, and Other Vasculitides 166
1. Introduction 166
2. Classification 166
3. Henoch-Schönlein purpura 166
4. Polyarteritis nodosa 170
5. Wegener granulomatosis 174
6. Takayasu arteritis 176
7. Other vasculitides 177
References 179
Chapter 13. Pediatric Antiphospholipid Syndrome 184
1. Introduction 184
2. Prevalence of aPL 184
3. Epidemiology of APS 186
4. Etiology and pathogenesis 187
5. Clinical manifestations 188
6. Diagnostic investigations 190
7. Differential diagnosis 191
8. Treatment 191
References 192
Chapter 14. Behçet’s Disease 196
1. Introduction 196
2. Epidemiology 196
3. Etiology/pathogenesis 197
4. Clinical manifestations 198
5. Diagnostic investigations 199
6. Differential diagnosis 200
7. Treatment 200
References 201
Chapter 15. Childhood Sarcoidosis 204
1. Introduction 204
2. Epidemiology 206
3. Etiology/pathogenesis 206
4. Clinical manifestations 210
5. Diagnostic investigations 216
6. Differential diagnosis 217
7. Treatment and prognosis 219
References 220
Chapter 16. Rheumatic Fever 224
1. Introduction 224
2. Prevalence 224
3. Epidemiology 224
4. Etiology/pathogenesis 224
5. Clinical manifestations 225
6. Diagnosis 227
7. Treatment 229
References 230
Chapter 17. Lyme Borreliosis 234
1. Introduction 234
2. Persistent infection 234
3. Immunopathological reactions 235
4. Autoimmunity 235
5. Conclusions 235
References 236
Chapter 18. TNF-Inhibitors in Pediatric Rheumatology 238
1. Introduction 238
2. TNF in the pathogenesis of JIA 239
3. Adverse events of TNFa blockade 243
4. TNFa-inhibitors in juvenile dermatomyositis 247
5. TNFa-inhibitors in other pediatric rheumatic diseases 247
6. Conclusions 248
References 249
Chapter 19. Autologous Stem Cell Transplantation (ASCT) for Drug Resistant Systemic Onset Juvenile Idiopathic Arthritis 256
1. Rational of autologous stem cell transplantation for autoimmune disease 256
2. Autologous stem cell transplantation for juvenile idiopathic arthritis 257
3. Clinical results 258
4. Mechanisms of tolerance induction by ASCT 259
Reference 262
Chapter 20. Physical Therapy Management of Pediatric Rheumatology Conditions 266
1. Physical therapy management 266
2. Information to be gained from the subjective assessment 266
3. Objective assessment 271
4. Physical therapy management 273
References 280
Subject Index 284
Colour Plate Section 290