Pediatric Rheumatology in Clinical Practice

304 Pages·2016·4.777 MB·English

by  Ronald M. Laxer, David D. Sherry, Philip J. Hashkes (auth.)

Ronald M. Laxer · David D. Sherry
Philip J. Hashkes
Pediatric Rheumatology
in Clinical Practice
Second Edition
123
Pediatric Rheumatology
in Clinical Practice

Ronald M. Laxer (cid:129) David D. Sherry
Philip J. Hashkes
Pediatric Rheumatology
in Clinical Practice
Second Edition
Ronald M. Laxer Philip J. Hashkes
Hospital for Sick Children Pediatric Rheumatology Unit
University of Toronto Shaare Zedek Medical Center
Toronto , Ontario Jerusalem
Canada Israel
David D. Sherry
Children’s Hospital of
Philadelphia
Philadelphia , PA
USA
ISBN 978-3-319-13098-9 ISBN 978-3-319-13099-6 (eBook)
DOI 10.1007/978-3-319-13099-6
Library of Congress Control Number: 2016941878
© Springer-Verlag London 2016
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Pref ace
Pediatric rheumatology is a relatively new specialty, with
many fascinating conditions peculiar to young people, virtually
all of which are considered “orphan” diseases that are only
rarely encountered by the generalist and therefore often pres-
ent as diagnostic and therapeutic dilemmas. The popularity of
the initial edition of this handbook suggested the need for a
resource to fill the knowledge gap by assisting the pediatri-
cian, the general practitioner, as well as rheumatologists with
an interest in pediatric rheumatology, in the diagnosis and
management of these diseases and problems. The emphasis
has been placed on clinical presentation and how to arrive at
a diagnosis, and an up-to-date management plan, using tables,
algorithms, and figures as visual aids. Brief background infor-
mation on the etiology is also provided. In this second edition
we have added new knowledge accumulated since the first
edition was published in 2007, particularly on new treatments
for juvenile idiopathic arthritis and advances in the field of
autoinflammatory diseases. We have also added a new chapter
on tumors involving the musculoskeletal system. We sincerely
hope that the information herein will help decrease the
impact of these conditions on the children and their families
by timely diagnosis and early intervention.
Toronto , ON , Canada Ronald M. Laxer
Philadelphia , PA , USA David D. Sherry
Jerusalem , Israel Philip J. Hashkes

v

Contents
1 General Presentation of Musculoskeletal Problems
in Childhood. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
1.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1
1.2 Rheumatologic History. . . . . . . . . . . . . . . . . . . . 1
1.3 Family History . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
1.4 Social History. . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
1.5 Past History . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4
1.6 General Physical Examination . . . . . . . . . . . . . 4
1.7 Joint Examination . . . . . . . . . . . . . . . . . . . . . . . . 5
1.8 Laboratory and Imaging Studies. . . . . . . . . . . . 6
1.9 Clues to the Diagnosis . . . . . . . . . . . . . . . . . . . . 10
1.10 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
1.11 Prevalence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 20
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22
2 General Principles of Management. . . . . . . . . . . . . . 23
2.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 23
2.2 Inflammatory/Autoimmune Diseases. . . . . . . . 24
2.3 Pain in Inflammatory Diseases . . . . . . . . . . . . . 25
2.4 Multidisciplinary Approach to Management. . 27
2.5 Complementary and Alternative Therapies. . . 28
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 29
3 Juvenile Idiopathic Arthritis (JIA). . . . . . . . . . . . . . 31
3.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
3.2 Oligoarthritis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 32
3.2.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . 32
3.2.2 Epidemiology. . . . . . . . . . . . . . . . . . . . . . 32
3.2.3 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . 33
3.2.4 Clinical Manifestations . . . . . . . . . . . . . . 33
3.2.5 Laboratory Features . . . . . . . . . . . . . . . . 35
vii
viii Contents
3.2.6 Establishing the Diagnosis . . . . . . . . . . . 35
3.2.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 37
3.2.8 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . 40
3.3 Polyarthritis, Rheumatoid Factor (RF)
Negative . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
3.3.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . 41
3.3.2 Epidemiology. . . . . . . . . . . . . . . . . . . . . . 41
3.3.3 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . 41
3.3.4 Clinical Manifestations . . . . . . . . . . . . . . 41
3.3.5 Laboratory Features . . . . . . . . . . . . . . . . 41
3.3.6 Establishing the Diagnosis . . . . . . . . . . . 42
3.3.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 42
3.3.8 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . 42
3.4 Polyarthritis, Rheumatoid Factor (RF)
Positive . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 44
3.4.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . 44
3.4.2 Epidemiology. . . . . . . . . . . . . . . . . . . . . . 44
3.4.3 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . 44
3.4.4 Clinical Manifestations . . . . . . . . . . . . . . 44
3.4.5 Laboratory Features . . . . . . . . . . . . . . . . 45
3.4.6 Establishing the Diagnosis . . . . . . . . . . . 45
3.4.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 45
3.4.8 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . 46
3.5 Systemic JIA (sJIA) . . . . . . . . . . . . . . . . . . . . . . 46
3.5.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . 46
3.5.2 Epidemiology. . . . . . . . . . . . . . . . . . . . . . 46
3.5.3 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . 46
3.5.4 Clinical Manifestations . . . . . . . . . . . . . . 47
3.5.5 Laboratory Features . . . . . . . . . . . . . . . . 48
3.5.6 Establishing a Diagnosis. . . . . . . . . . . . . 49
3.5.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 50
3.5.8 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . 50
3.6 Enthesitis-Related Arthritis (ERA) . . . . . . . . . 52
3.6.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . 52
3.6.2 Epidemiology. . . . . . . . . . . . . . . . . . . . . . 52
3.6.3 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . 52
3.6.4 Clinical Manifestations . . . . . . . . . . . . . . 53
3.6.5 Investigations . . . . . . . . . . . . . . . . . . . . . . 54
3.6.6 Establishing a Diagnosis. . . . . . . . . . . . . 54
Contents ix
3.6.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 56
3.6.8 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . 57
3.7 Psoriatic Arthritis . . . . . . . . . . . . . . . . . . . . . . . . 57
3.7.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . 57
3.7.2 Epidemiology. . . . . . . . . . . . . . . . . . . . . . 57
3.7.3 Etiology. . . . . . . . . . . . . . . . . . . . . . . . . . . 58
3.7.4 Clinical Manifestations . . . . . . . . . . . . . . 58
3.7.5 Laboratory Features . . . . . . . . . . . . . . . . 59
3.7.6 Establishing the Diagnosis . . . . . . . . . . . 59
3.7.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . 59
3.7.8 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . 60
3.7.9 Unclassified Arthritis. . . . . . . . . . . . . . . . 60
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60
4 Systemic Lupus Erythematosus. . . . . . . . . . . . . . . . . 63
4.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 63
4.2 Etiology and Pathogenesis. . . . . . . . . . . . . . . . . 63
4.3 Epidemiology. . . . . . . . . . . . . . . . . . . . . . . . . . . . 65
4.4 Clinical Manifestations. . . . . . . . . . . . . . . . . . . . 65
4.4.1 Mucocutaneous . . . . . . . . . . . . . . . . . . . . 66
4.4.2 Musculoskeletal . . . . . . . . . . . . . . . . . . . . 70
4.4.3 Neurologic. . . . . . . . . . . . . . . . . . . . . . . . . 72
4.4.4 Serositis. . . . . . . . . . . . . . . . . . . . . . . . . . . 73
4.4.5 Renal Disease. . . . . . . . . . . . . . . . . . . . . . 73
4.4.6 Antiphospholipid
Antibody Syndrome (APLS). . . . . . . . . 74
4.4.7 Hematologic. . . . . . . . . . . . . . . . . . . . . . . 75
4.4.8 Cardiac . . . . . . . . . . . . . . . . . . . . . . . . . . . 76
4.4.9 Pulmonary. . . . . . . . . . . . . . . . . . . . . . . . . 76
4.4.10 Gastrointestinal . . . . . . . . . . . . . . . . . . . . 76
4.4.11 Sicca Syndrome . . . . . . . . . . . . . . . . . . . . 77
4.4.12 Endocrine . . . . . . . . . . . . . . . . . . . . . . . . . 77
4.5 Laboratory Abnormalities . . . . . . . . . . . . . . . . . 77
4.6 Making the Diagnosis. . . . . . . . . . . . . . . . . . . . . 78
4.7 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 79
4.8 Prognosis/Outcome. . . . . . . . . . . . . . . . . . . . . . . 82
4.9 Neonatal Lupus Erythematosus (NLE). . . . . . 85
4.10 Drug Induced Lupus. . . . . . . . . . . . . . . . . . . . . . 86
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88