ABSTRACT

Preface . . . . . . . . . . . . . . . . 5 Acknowledgments . . . . . . . 6

CHAPTER 1 Introduction Thomas Mason Assessment of the child with rheumatic disease Approach to children with rheumatic disease Inflammatory versus

non-inflammatory . . . . . . 8 Acute, inflammatory,

articular . . . . . . . . . . . . . . 8 Chronic, inflammatory,

articular . . . . . . . . . . . . . . 8 Chronic, inflammatory,

organ involvement . . . . . 10

CHAPTER 2 Juvenile idiopathic arthritis Egla Rabinovich and Angela Robinson Systemic arthritis . . . . . . . . . 14 Oligoarthritis . . . . . . . . . . . 21 Polyarthritis . . . . . . . . . . . . 28

spondyloarthropathy . . . 38 Juvenile ankylosing

spondylitis . . . . . . . . . . . 42 Juvenile psoriatic

arthritis . . . . . . . . . . . . . 48 Inflammatory bowel

disease-associated arthropathy . . . . . . . . . . 50

Reactive arthropathies Reactive arthritis . . . . . . . . . 54

Post-streptococcal arthropathies Acute rheumatic fever . . . . . 56 Post-streptococcal reactive

arthritis . . . . . . . . . . . . . 58

erythematosus . . . . . . . . 62 Mixed connective tissue

disease . . . . . . . . . . . . . . 76 Neonatal lupus

syndrome . . . . . . . . . . . . 80 Cutaneous lupus

erythematosus . . . . . . . . 82

CHAPTER 5 Idiopathic inflammatory myopathies Floranne Ernste and Ann M Reed Juvenile dermatomyositis . . 88 Juvenile polymyositis . . . . . 101 Postinfectious myositis . . . 103

CHAPTER 6 Vasculitis Robert Sundel Kawasaki disease . . . . . . . . 106 Henoch-Schönlein

pupura . . . . . . . . . . . . . 109 Polyarteritis nodosa . . . . . . 111 ANCA-associated

vasculitides . . . . . . . . . . 114 Takayasu arteritis . . . . . . . 118 Primary angiitis of

the central nervous system . . . . . . . . . . . . . 120

sclerosis . . . . . . . . . . . . 124 Juvenile localized

scleroderma . . . . . . . . . 130

fever . . . . . . . . . . . . . . 140 Cold-induced

autoinflammatory syndrome 1 (CIAS1-pathies) – the cryopyrin-associated periodic fever syndromes . . . . . . . . . . 144

Hyperimmunoglobulinemia D with periodic fever syndrome . . . . . . . . . . . 146

Tumor necrosis factor receptor-associated periodic syndrome . . . . 148

Other monogenic autoinflammatory syndromes

Pyogenic sterile arthritis, pyoderma gangrenosum, and acne syndrome . . . . 150

Early onset sarcoidosis (sporadic granulomatous arthritis), Blau syndrome (familial granulomatous arthritis), and Crohn disease . . . . . . . . . . . . . 150

Autoinflammatory syndromes that are not monogenic/ autoinflammatory diseases with complex genetic traits . . . . . . . . 154