Pediatric Rheumatology for the Practitioner Second Edition

Jerry C. Jacobs

Pediatric Rheumatology for the Practitioner Second Edition

With 330 Illustrations in 422 Pieces

Springer Science+Business Media, LLC

Jerry C.Jacobs, M.D. Professor of Clinical Pediatrics Director, Section ofPediatric Rheumatology

and the Regional Arthritis Program (RAP-4-Kids) at the

Columbia-Presbyterian Medical Center New York, NY 10032 USA

Library ofCongress Cataloging-in-Publication Data Jacobs,Jerry C.

Pediatric rheumatology for the practitioner 1 Jerry C.Jacobs.-2nd ed.

p. cm. Includes bibliographical references and index. ISBN 978-1-4757-6152-8 ISBN 978-1-4757-6150-4 (eBook) DOI 10.1007/978-1-4757-6150-4

1. Pediatric rheumatology. I. Title. [DNLM: 1. Collagen Diseases-in infancy & childhood.

Jl7p] RJ482.R48J3 1992 618.92'723-dc20 DNLM/DLC for Library ofCongress

Printed on acid-free paper.

© 1982 © 1993 Springer Science+Business Media New York Originally published by Springer-Verlag New York, Inc. in 1993

WD375

91-5210

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Production managed by Ellen Scham; Manufacturing supervised by Rhca Talbcrt. Typeset by Asco Trade Typcsctting Ltd., Hong Kong.·

9 8 7 6 5 4 3 2 1

ISBN 978-1-4757-6152-8

To my wife Isabel, who has provided very special inspiration for 42 years;

and to Deborah, Daniel, and Paul, who added another dimension to my

life and sometimes sacrificed their own needs to those of other children.

To all my teachers, but especially to Gilbert Hiatt and Charles Frankel, who

taught a generation of Columbia College students about life; to Robert

Loeb, Dana Atchley, and Rustin Mcintosh, who taught a generation of

physicians about devotion to the care of the sick; to Charles Ragan, who

convinced me to study rheumatic disease; and to Charles Christian who

provided my training and inspiration.

To all the sick children and their families, who are the substance of this book

and for whom we hope to do more.

"To study the phenomena of disease without books is to sail an uncharted sea, while to study books without patients is not to go to sea at all."

-W. Osler ("Books and Men," Boston Medical and Surgicaljoumal, 144:60-61, 1901)

Contents

Preface to the Second Edition xvn

Preface to the First Edition x1x

Acknowledgments xxm

1 Clinical Techniques in Pediatric Rheumatology Taking the History 1 Physical Examination 5 Evaluation of the Functional State of the Patient 19 Laboratory Procedures 21

Radiographic Techniques in Childhood Rheumatic Disease 22

2 The Differential Diagnosis of Arthritis in Childhood 25 Acute Rheumatic Conditions 26

Acute Rheumatic Fever 27 Poststreptococcal Reactive Arthritis 31 Henoch-Schonlein Purpura 32 Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) 33 Erythema Nodosum 33 Serum Sickness and Drug Reactions 34 Mucha-Habermann Disease (PLEVA) 36 Sweet's Syndrome 37 Thrombotic Thrombocytopenic Purpura (TTP) 40

Acute and Chronic Infections of the Bones and joints 40 Acute Hematogenous Osteomyelitis 40 Osteomyelitis in the Sacroiliac Area 49 Subacute Osteomyelitis 49

Puncture Wounds of the Feet 51

VII

Vlll Contents

Subacute Osteomyelitis of the Patella 52 Suppurative Prepatellar Bursitis 52 Chronic Osteomyelitis 53 Special Situations-Suppurative Iliac Lymphadenitis and Retroperi-

toneal (Psoas) Abscess 54 Pyomyositis 57 Hemophilus injluenzae Cellulitis of the Hand 59 Diskitis 59 Chronic Recurrent Multifocal Osteomyelitis (CRMO) 63 Tuberculosis 65 Brucellosis 65 Salmonellosis 65 Cat-Scratch Disease 66 Acute Septic Arthritis (Nongonococcal) 66 The Gonococcal Arthritis-Dermatitis-Tenosynovitis Syndrome 67 Foreign-Body Synovitis 68 Fungal Arthritis 71 Parasitic Arthritis 7l Syphilis and Other Diseases Caused by Flexibacteria 71 Viral Arthritis 72

Rubella: The "Catcher's Crouch" Syndrome 73 Hepatitis 74 Parvovirus B 19 7 4

Chronic Active Liver Disease (CALD) 75 Arthritis Associated with Other Infectious Agents 75 Arthropod-borne Arthritis 76

Rocky Mountain Spotted Fever 76 Lyme Disease 76

Special Syndromes Related to Infection 83 Musculoskeletal Manifestations ofBacterial Infection 83 Arthritis as a Manifestation of Subacute Bacterial Endocarditis 83 Shunt Arthritis 83 Acne Rheumatism 83 Arthritis and Rash after Intestinal Bypass Surgery 84 Whipple's Disease 84 The Phalangeal Microgeodic Syndrome 85 AIDS 86 Rheumatic Manifestations of Substance Abuse 86

Inflammatory Disorders 86 Transient Synovitis of the Hip 86 Inflammatory Bowel Disease (IBD) with Arthritis 87 Familial Mediterranean Fever (FMF) 89 Solitary Mastocytoma with Flushing 95 Relapsing Polychondritis 96 Muckle-Wells Syndrome 101

Contents IX

Idiopathic Periosteal Hyperostosis (Goldbloom's Syndrome) 101 Weber-Christian Disease 102 Acute Pancreatitis with Arthritis 103 Pseudotumor of the Orbit 103 Cortical Hyperostoses 105

Caffey's Disease (Infantile Cortical Hyperostosis) 105 Melorheostosis 105

Intramedullary Osteosclerosis 105 Diaphyseal Osteosclerosis 106 Sarcoid Arthritis 107 Pseudorheumatoid Nodules 109 Transient Hyperphosphatasemia oflnfancy 109 Erythromelalgia 111

Hypertrophic Osteoarthropathy 111 Secondary Hypertrophic Osteoarthropathy 111

Cystic Fibrosis (CF) 112 Celiac Disease 113 Arthritis Associated with Absence of the Bile Ducts 113

Alagille's Syndrome 113 Pseudoarthritis Associated with Congenital Absence oflntrahepatic

Bile Ducts 115 Malignant Disease in Childhood Presenting with Musculoskeletal

Pain 115 Primary Hypertrophic Osteoarthropathy 117

Pachydermoperiostosis 117 Familial HO without Pachyderma 117

Unusual Traumas and Unusual Responses to Trauma 118 Stress Fractures 118 Acute Chondrolysis of the Hip (Juvenile Lamellar Coxitis) 120 Transitory Demineralization of the Hips 122 Special Forms ofTrauma 122

Child Abuse 122 Congenital Indifference to Pain (Charcot's joints in Childhood) 126 Frostbite 127 Eosinophilic Synovitis 128 Erythrocyte and DNA Autosensitivity 128

Degenerative Disorders 128 Avascular ("Aseptic") Necrosis ofBone (AVN) 128 CrackingJoints 131 Chondromalacia Patellae 131 Osteochondritis Dissecans 132 Slipped Capital Femoral Epiphysis 132 Osteoarthritis in Childhood 133

Metabolic Diseases 133 Diabetic Cheiroarthropathy (Diabetic Hand Syndrome) 134

X Contents

Disorders of Bone Metabolism 136 Idiopathic Juvenile Osteoporosis 136 Hyperparathyroidism 138 Dialysis Arthritis 138 Late-Onset Rickets 138 Endocrinopathies with Arthritis 139 Hypothyroidism 139 Hyperthyroidism 140 Cushing's Syndrome and Addison's Disease 140

Vitamin and Fluoride Poisoning 141 Vitamin A Poisoning 141 Vitamin D Poisoning 142 Fluorosis 142

Familial Hyperlipoproteinemia 143 Type Ila Hyperlipoproteinemia 143 Type IV Hyperlipoproteinemia 143

Sitosterolemia 143 Gout 143 Hyperuricemia and Secondary Gout 146 Lowe's (Oculocerebrorenal) Syndrome 147 Williams (Elfin Facies) Syndrome 147 Alcaptonuria 14 7 Hemochromatosis 14 7 Kashin-Beck Disease (Urov Disease) 148 MseliniJoint Disease 149 Calcific Periarthritis 149

Abnormal Blood Proteins 149 Hemoglobinopathies-Sickle-Cell Disease 149 Hemophilias and Thrombasthenias 150 Immunologic Deficiency Disorders with Arthritis 151 Familial Lipochrome Histiocytosis 152 Arthritis, Pyoderma Gangrenosum, and Streaking Leukocyte

Factor 153 Waldenstrom's Hypergammaglobulinemic Purpura 157 Hereditary Angiodema (HAE) 158 Cold Fibrinogenemia 158 Arthritis as a Manifestation of Food Allergy 158

Heritable Disorders Associated withJoint Laxity 159 Marfan's Syndrome 159 Ehlers-Danlos Syndrome (EDS) 160 Benign Hypermobility 161 Down's Syndrome (Trisomy 21) 163 Larsen's Syndrome 163

Joint Contractures at Birth 164 Nail-Patella Syndrome (Onycho-osteo-arthro Dysplasia) 164

Contents xi

Arthrogryposis Multiplex Congenita 164 Fetal Alcohol Syndrome 165 Congenital Contractual Arachnodactyly 165 Chromosomal Abnormalities 165 Punctate Epiphyseal Dysplasia 165 Congenital joint Contractures with Facial Abnormalities 165 The Stiff-Skin Syndromes 166

Hypomobile (Stiff-Joint) Syndromes Appearing after Birth 166 The Mucopolysaccharidoses and Mucolipidoses 167 The Weill-Marchesani Syndrome 173 Leri's Pleonosteosis 173 Myositis Ossificans (Fibrodysplasia Ossificans Progressiva) 173 Stickler Syndrome 173 Multicentric Reticulohistiocytosis 174 The Lipidoses 174

Gaucher's Disease 174 Fabry's Disease 174 Ceramidosis (Farber's Disease) 175

Bone and Cartilage Dysplasias 1 77 Trichorhinophalangeal Dysplasia (TRPS) 177 Multiple Cartilaginous Exostoses 178 Metachondromatosis 178 The Vanishing Bone Diseases 180

Carpotarsal Osteolysis 180 Winchester Syndrome 181 Congenital Arthropathy with Rash Appearing at Birth; Infantile Multi­

system Inflammatory Disease (CINCA, NO MID Syndrome) 182

Infantile Systemic Hyalinosis 182 Familial Hypertrophic Synovitis 183

Neoplasms and Neoplasmlike Lesions of the Bones andjoints 186 Tumors ofBone 186 Osteoid Osteoma 186 Benign Neoplasms of the joints 189

Ganglions 189 Synovial Hemangiomas 190 Pigmented Villondular Synovitis 191 Synovial Chondromatosis 193 Langer hans' -cell Histiocytosis 193

Malignant Synovial Tumors 193 Synovial Sarcoma 193 Epithelioid Sarcoma 194 Epithelioid Hemangioendothelioma 194

Psychiatric Disorders 196 Is the Disease "Organic" or "Functional"? 196

xii Contents

Psychogenic Rheumatism 196 Fibrositis/Fibromyalgia/Tension Myalgia 197 Reflex Sympathetic Dystrophy 198 Chronic Fatigue Syndrome 202 Munchausen's Syndrome and Factitious Fever

3 Juvenile Rheumatoid Arthritis Diagnostic Criteria 231 Subtypes of]RA 231 Laboratory Studies 236 Radiographic Studies 240 Epidemiology of Arthritis in Childhood 240 Familial Incidence 241 SystemicJRA (Still's Disease) 241

Prevention of Death 256 Recognition ofDIC inJRA 256 Prevention of Addisonian Crisis 257

Polyarticular Juvenile Rheumatoid Arthritis Pauciarticular Juvenile Rheumatoid Arthritis Iridocyclitis (Anterior Uveitis) 314 Arthritis with Psoriasis 318 Exercises as Therapy in Childhood Arthritis Surgery inJRA 324

273 311

320

202

4 HLA-827-Associated Spondyloarthritis and Enthesopathy

Historical Considerations 361 The Histocompatibility System and Disease 365 HLA-B27 and Arthritis 368

Ankylosing Spondylitis 368 Reiter's Syndrome and "Reactive Arthritis" 369

HLA-B27-Associated Spondyloarthritis and Enthesopathy 371

231

360

The Clinical Presentation of Children with B27-Associated Arthritis 372 Course of the Disease 391 Treatment 399

5 Systemic Lupus Erythematosus 409 Prologue 409 Epidemiology and Susceptibility 410 Immune Complexes and the Pathophysiology ofSLE 415 Clinical Measurement of Serum Immune Complexes, Complement, and

Anti-DNA Antibodies 416 Tests for Antinuclear Antibodies and the Diagnosis ofSLE 419

Contents xiii

The Pathology ofSLE 422 Estimating Prognosis in SLE 430 Causes of Death in SLE 432 Spontaneous Remission in SLE 432 The Clinical Picture ofSLE in Childhood 433

Presenting Manifestations 433 Diagnosis 434

Clinical Manifestations 435 SLE and Pregnancy 473 Lupus in the Neonate 474

Onset ofLupus in the First Year ofLife 476 Special Lupus Erythematosus Syndromes 476

Drug Induced Lupus 476 Mixed Connective Tissue Disease 480 Discoid LE 482

Treatment ofSLE in Children 484 Goals ofTherapy 484 Principles of Therapy 484 Management Techniques 490

6 Dermatomyositis 527 Clinical Presentation 527 Immunopathology 528 Epidemiology 529 Clinical Features 530 Course of the Disease 542 Management and Prognosis 544 Disorders Resembling Dermatomyositis 545

Inclusion Body Myositis 545 Transient Acute Viral Myositis 546 Trichinosis and Hypereosinophilic Syndromes 547 Steroid Myopathy 547 Persistent and Fatal Central Nervous System Echovirus Infections in

Patients with Agammaglobulinemia 54 7 Pitfalls in Interpreting Serum CPK Activity 548 Hypothyroidism 548 Rhabdomyolysis and Lipid Storage Myopathy 548 Dermatomysitis and Malignancy 549 Muscular Dystrophy 549 Toxoplasmosis 549 Drug-Induced Dermatomyositis 549 Granulomatous Myositis 550 Chronic Congenital Myopathy Associated with Coxsackie

Virus A9 550 Calcinosis and Dermal Bone-Forming Disorders 550

xiv Contents

7 Systemic Vasculitis Syndromes 556 Clinical Presentation 556

Kawasaki Disease 559 Polyarteritis Nodosa 583 Allergic Angiitis and Granulomatosis (Churg-Strauss Syndrome) 588 Primary Granulomatous Angiitis of the Brain 589 Steroid-Responsive Encephalomyelitis (Allergic Angiitis of the

Brain) 591 Takayasu's Arteritis 592 Familial Granulomatous Arteritis 596 Wegener's Granulomatosis 596 Limited Wegener's Granulomatosis 599 Lymphomatoid Granulomatosis 599 Hypersensitivity Vasculitis 602 Cutaneous Vasculitis 608 Beh~et's Syndrome 610 Cogan's Syndrome 613 Malignant Atrophic Papulosis (Kohlmeier-Degos Disease) 615 Juvenile Temporal Arteritis 615 Disorders Simulating Systemic Vasculitis 616

Left Atrial Myxoma 618 Hereditary Disorders of Amino-Acid Metabolism Simulating Systemic

Vasculitis 617 Primary Hyperoxaluria 618 Pheochromocytoma 618 Hypercoagulable States 619 MELAS Syndrome 619 Moyamoya 619 Hemiplegic Migraine 621 Goodpasture's Syndrome 621 Toxic Shock Syndrome 622

8 Scleroderma Clinical Presentation Immunopathology Diagnosis 645 Epidemiology 647 Classification 647

641 644

Clinical Features 648 Course of the Disease 655 Treatment 657 Disorders Resembling Scleroderma 659

Scleredema (The China-Doll Syndrome) 660

641

Subcutaneous Lipogranulomatosis (Rothmann-Makai Syndrome) 661

Contents XV

Eosinophilic Fasciitis 663 Toxic Oil Syndrome and Eosinophilia-Myalgia Syndrome Induced by

"Toxic" L-Tryptophan 667 Iatrogenic Fibrous Myopathy 669 Drug-Chemical-Induced Scleroderma-like Disease 669 Sclerodermalike Syndrome as Part of Chronic Cutaneous Graft-Versus-

Host Reaction 669 Scleroderma Syndromes as Part of Human Adjuvant Disease 670 Scleromyxedema 670 Parry-Romberg Syndrome (PRS) 671

9 The Power of Positive Thinking 678 Coping with Chronic Illness 681 Helping the Family Cope with Sickness 682 The Importance of Motor Activity in the Personality Development of

Children 684 The Importance of Physician Attitudes in the Care of the Chronically

Ill 686 Society's Attitudes Toward the Crippled 687 School and the Crippled Child 689 Sex and the Disabled 690 Psychosocial Concepts in the Etiology ofRA 691 Psychotherapy in the Care of Chronically Ill Children 692 Group Psychotherapy for Sick Children and Their Parents 693 Vocational Education for the Handicapped 694 Staying in the Mainstream 695

Index 701

Preface to the Second Edition

Exactly ten years have elapsed since I wrote the preface to the first edition; pediatric rheumatology has grown into an accepted specialty and this year 103 physicians applied to take the first sub-Board examination. The exten­sive discussion of differential diagnosis required even further expansion: post­streptococcal arthritis, thrombotic thrombocytopenic purpura, Parvo 19, Muckle-Wells syndrome, Weber-Christian disease, transient hyperphospha­tasemia of infancy, erythromelalgia, primary hyperoxaluria, fibromyalgia, sito­sterolemia, AIDS, arthritis as a manifestation of substance abuse, HLA-B27 associated familial thrombocytopenia with spondyloarthritis, Schmidt's syn­drome, the anticardiolipin syndrome, inclusion body myositis and the toxic oil and L-tryptophan induced eosinophilia-myalgia and Parry-Romberg syn­dromes are only some of the added sections. The discussion of Lyme disease, the most common identifiable cause of arthritis in the United States, has been greatly expanded.

What has changed most in ten years, however, is drug treatment of the rheumatic disorders. Non-steroidal antiinflammatory drugs (NSAIDs) have replaced aspirin as the standard treatment of childhood arthritis, and methotrexate, not previously used at all, has become the drug of choice for the most severely ill children. Sulfasalazine, also not previously used at all, is now the most commonly used "disease modifying" agent in our clinic, and the last gold shot given to a child here was in 1986. Sections on preventing death from disseminated intravascular coagulation (DIC) and on how to better control fever in systemic JRA have been added. The severe cardiac sequelae of Kawasaki disease, the most common cause of acquired heart disease in U.S. children 10 years ago, have been almost totally prevented by early diagnosis and prompt administration of gamma globulin. And we have learned that severe lupus nephritis needs even more aggressive treatment than we previously offered if renal function and life are to be preserved. We've failed only once in preventing a child from becoming wheelchair

xvii

XVlll Preface to the Second Edition

bound from arthritis in this decade, and I believe we could have done better for him too, and we've had no deaths in JRA or dermatomyositis or renal failure or deaths in Caucasian children with lupus. Until I had completed writing this edition I did not realize how much had changed.

Jerry C. Jacobs

Preface to the First Edition

Definition of the Rheumatic Disorders: "A typical hippie commune, a hitherto forbid­den clone, sharing a mystery of origin, living together but unrelated, widely mis­understood, difficult to control or to treat, error-prone, over-reactive sometimes to familiar antigens, to parental influence and to medical authority, given to strange drugs, difficult to recognize from each other and adding a few more mixed-up syn­dromes to their number from time to time ... "*

This book complements the authoritative multiauthored texts on rheumatic disease by providing special insight into the care of children with rheumatic disease. Rheumatologists, orthopedists, and physiatrists are familiar with the principles of diagnosis and management of rheumatic diseases in adults, and they are called upon to apply their special knowledge to the care of children. Their devotion to young patients is evidenced by their attendance in over­whelming numbers at the relatively few seminars pediatric rheumatologists have been able to provide. My goal is to help them sharpen their diagnostic and therapeutic strategies. If each gains even a little from reading this book, the children will benefit, and I know that their doctors will obtain greater satisfaction from caring for them.

Inevitably in a one-author text whose primary purpose is to provide information to family practitioners and pediatricians, opinions will be ex­pressed which may seem arbitrary to specialized physicians. In some in­stances my knowledge of orthopedics, internal medicine, and physiatry must seem a bit primitive to real experts in these individual disciplines. It is not necessary, however, that we always agree. What I have written is based

*Paraphrased from Eric Bywaters, at the VIIIth European Congress of Rheumatolo­gy, Helsinki, Finland, june I, 1975. See Bywaters EGL: The historical evolution of the concept of connective tissue disease. Scandinavianjournal ofRheumatology. Supple­ment 12:11-29, 1976.

xix

XX Preface to the First Edition

largely on personal experience, and where the knowledgeable physician dis­agrees, it is hoped he will find our private debate stimulating.

For those whose primary mission is the care of children, my purposes are quite different. When invited by the Editors to contribute this volume to a new series ofmonographs entitled "Comprehensive Manuals in Pediatrics," I made a firm commitment to provide primary care physicians with the mul­tiple perceptions which are part of my clinical judgment in the diagnosis and care of children with musculoskeletal disorders. It is not my intention to provide an encyclopedic text. The reader is encouraged, whenever the oppor­tunity presents, to consult the many outstanding sources of rheumatologic information.

In the aggregate, the disorders discussed in this book inflict a considerable burden on children. Musculoskeletal disease accounts for nearly 2 percent of all visits to pediatricians and is one of the most common causes of chronic disability. Despite the evident need, relatively few medical schools have divi­sions of pediatric rheumatology. As a result, most pediatricians in practice today have had scanty training in the diagnosis and management of rheuma­tic disorders, or even in physical examination of the joints. The differential diagnosis of musculoskeletal pain in childhood is among the most complex of any in medicine. More than one hundred entities must be considered. For this reason, fully one-third of this book is devoted to differential diagnosis. The "nonrheumatic disorders," traditionally relegated to the back of ency­clopedic texts, have been brought "up front." With only a little help, every primary care physician for children can become more expert in differential diagnosis and can obtain great satisfaction from having developed these skills.

Diagnosis and treatment are the focus of the chapters on the specific rheumatic diseases. Theories about disease causation are presented only to whet the appetite. The principles of treatment have been emphasized, and in every instance I have tried to provide the physician with a plan of therapy which is acceptable, albeit at times controversial. In the ideal world, every child with rheumatic disease would have the benefit of the care of a full-time pediatric rheumatologist. Such a world does not now and will not soon exist. Pediatricians find it hard enough to work out a therapeutic strategy for these children without becoming embroiled in all the controversy on which rheumatologists thrive. It is well known that on rheumatology rounds expert physicians rarely advocate an identical plan of therapy for a given patient. A dialogue takes place from which the responsible individual must synthesize his plan of action. I have tried to provide an acceptable plan for primary physicians who do not have the opportunity to participate in this dialogue but are faced with the responsibility of caring for the patient.

Most pediatric rheumatologists already know much of the material in this book. We are a small group and exchange ideas frequently. I hope they find a few tidbits, but one can hardly justify writing a book for pediatric rheumato­logists. For the pediatric rheumatologist, perhaps the most controversial of

Preface to the First Edition xxi

all the concepts contained in this book is my suggestion that Reiter's syn­drome and ankylosing spondylitis are prototype descriptions of different stages of a single disease, which might best be called spondyloarthritis and which in many or most individuals does not affect the spine. This formulation greatly simplifies the nomenclature, since, otherwise, new names must be created for the majority of patients with this form of arthritis who have neither the Reiter's triad nor ankylosis of the spine, and are unlikely to develop either despite their increased susceptibility to both. The historical terms Reiter's syndrome and ankylosing spondylitis may be retained for \;\'hat they originally meant, without endless modification, and the same term may be used for the same disorder in both children and adults. An entire chapter is devoted to this relatively unstudied childhood disorder, which affects five times as many children as SLE, dermatomyositis, and scleroder­ma combined.

I cannot emphasize enough the importance of positive physician attitudes in minimizing rather than creating dysfunction. Over the past twenty years we have made relatively little progress in terms of new "curative" drugs or techniques or in the final understanding of the pathogenesis of any of these diseases. Yet any comparative study of the function of these children and their families in society shows dramatic advances. This is the triumph of pediatric rheumatology. Reduced hospitalization, little school absence, no special (but unequal) classes, less destruction of the family-these are our achievements. All have been accomplished primarily with the same old tools that were available before. The commitment to function, coupled with early skillful diagnosis and intervention, has enabled us to use both new and old drugs more effectively and to avoid modes of therapy which enhance dysfunc­tion. Pediatric rheumatologists didn't invent functional attitudes, but perhaps nowhere are they more crucial than in the care of visibly crippled children, and so perhaps their significance is most dramatically demon­strated in our patients.

Rheumatology has been largely a stepchild of medicine, devoid of great discoveries. The study of rheumatic diseases is fascinating but frustrating. We have had to settle for stimulating immunologic research, identifying and describing subsets of disease, establishing diagnostic criteria, designing ther­apeutic trials, and setting standards for exemplary care of patients. Preven­tion and cure remain as challenges for those who now choose pediatric rheumatology for their career. Hopefully, medical students and house officers who use this book will be encouraged to consider pediatric rheumatology as a potential career. If this book stimulates young doctors to study and improve the care of children with rheumatic diseases, it will have more than served its purpose.

Jerry C. jacobs

Acknowledgments

It is not possible to thank individually everyone who has contributed to the preparation of this book. Much has been learned from the children who suffer with these diseases and from their parents. Medical students, house officers, nurses, social workers, therapists, and clinic personnel, together with all of my physician colleagues at the Columbia-Presbyterian Medical Center, make up the team for the care of these patients and I welcome this opportu­nity to express my admiration and appreciation to all of them.

Dr. Walter Berdon contributed many, many hours to the selection and interpretation of the radiographs; most of these were photographed for pub­lication by Michael Carlin; almost all in Chapter 5 were photographed by Edward R. Hajjar. Drs. H. Joachim Wigger, Austin D. Johnston, David N. Silvers, Conrad L. Pirani and William A. Blanc provided the pathologic photomicrographs and their interpretation. Many of the clinical photographs were taken by the late Harry Preston and Grace MacMullen and by Bill Kramer. Bob Masini took some of the recent photographs. John W. Karapelou drew all of the wonderful figures.

The first edition manuscript was typed by Loretta Henke, one of the world's leading experts in interpreting poor handwriting.

I would like to thank, in particular, the late Larry Carter for his con­tinuous encouragement, which was badly needed. I am grateful to all of these individuals and to many others for their help and for their friendship.

xxiii

Pediatric Rheumatology for the Practitioner Second Edition