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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
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