Series Editors Michael Katz E. Richard Stiehm3A978-1-4757-6153-5%2F1.pdfFamilial Hypertrophic Synovitis 151 Neoplasms and Neoplasm-like Lesions of the Bones and Joints 155 Tumors of

e

~ III Comprehensive Manuals in Pediatrics Series Editors Michael Katz E. Richard Stiehm

Pediatric Rheumatology for the Practitioner

Jerry C. Jacobs Professor of Clinical Pediatrics Director, Section of Pediatric Rheumatology Department of Pediatrics College of Physicians and Surgeons Columbia University

Foreword by Charles L. Christian

With 406 Illustrations

Springer Science+Business Media, LLC

Jerry C. Jacobs, M.D.

Professor of Clinical Pediatrics Director, Section of Pediatric Rheumatology Department of Pediatrics CQllege of Physicians and Surgeons Columbia 'University New York, New York, U.S.A.

Series Editors Michael Katz, M.D.

Reuben S. Carpentier Professor and Chairman, Department of Pediatrics College of Physicians and Surgeons Columbia University and Director, Pediatric Service Presbyterian Hospital (Babies Hospital) New York, New York, U.S.A.

Production: William J. Gabello

E. Richard Stiehm, M.D.

Professor of Pediatrics UCLA School of Medicine UCLA Hospitals and Clinics Los Angeles, California, U.S.A.

Frontispiece: Twenty-two-month-old boy with characteristic JRA rash. After several years of polyarthritis and exacerbations of fever as high as 105.6°, the disease disappeared leaving disability (now age 14 years) only in the wrists.

Library of Congress Cataloging in Publication Data Jacobs, Jerry C.

Pediatric rheumatology for the practitioner. (Comprehensive manuals in pediatrics) Bibliography: p. Includes index. 1. Rheumatism in children. 1. Title. II. Series.

[DNLM: 1. Collagen diseases-In infancy and childhood. WD 375 J17rJ RJ482.R48J31982 618.92'723 82-10485

© 1982 by Springer Science+Business Media New York Originally published by Springer-Verlag New York Heidelberg Berlin in 1982 Softcover reprint of the hardcover 1 st edition 1982

AII rights reserved. No part of this book may be translated or reproduced in any form without written permission from Springer Science+Business Media, LLC

The use of general descriptive names, trade names, trademarks, etc. in this publication, even if the former are not especially identified, is not to be taken as a sign that such names, as understood by the Trade Marks and Merchandise Marks Act, may accordingly be used freely by anyone. While the advice and information in this book are believed to be true and accurate at the date of going to press, neither the author, nor the editors, nor the publisher can assume any legal responsibility for the information, especially as regards directions for dosage and form of application, given herein, or for any errors or omissions that may be made. The individual practitioner cannot rely exclusively on the information contained in this book, but must in every case make an independent judgment as to the requirements of an individual patient.

987654321

ISBN 978-1-4757-6155-9 ISBN 978-1-4757-6153-5 (eBook) DOI 10.1007/978-1-4757-6153-5

To my wife Isabel, who has provided very special inspiration for 32 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 Surgical Journal, 144:60-61,1901)

Contents

Series Foreword Michael Katz and E. Richard Stiehm

Foreword Charles L. Christian

Preface

Acknowledgments

Sources of Rheumatologic Information

1 Clinical Techniques in Pediatric Rheumatology Taking the History Physical Examination 5 Evaluation of the Functional State of the Patient 18 Laboratory Procedures 20

xv

XVll

XIX

XXlll

XXV

1

2 The Differential Diagnosis of Arthritis in Childhood 23 Acute Rheumatic Conditions 24

Acute Rheumatic Fever 24 Henoch-Schonlein Purpura 28 Kawasaki Disease (Mucocutaneous Lymph Node Syndrome) 30 Erythema Nodosum 30 Serum Sickness and Drug Reactions 31 Mucha-Habermann Disease 32 Sweet's Syndrome 34

VW Contents

Acute and Chronic Infections of the Bones and Joints 36 Acute Hematogenous Osteomyelitis 36 Osteomyelitis in the Sacroiliac Area 46 Subacute Osteomyelitis 46

Puncture Wounds of the Feet 48 Subacute Osteomyelitis of the Patella 48

Suppurative Prepatellar Bursitis 50 Chronic Osteomyelitis 50 Special Situations-Suppurative Iliac Lymphadenitis and

Retroperitoneal (Psoas) Abscess 51 Pyomyositis 52 Hemophilus injluenzae Cellulitis of the Hand 55 Diskitis 57 Stress Fractures 59 Chronic Recurrent Osteolysis without Infection 60 Tuberculosis 61 Salmonellosis 62 Cat-Scratch Fever 62 Acute Septic Arthritis (Nongonococcal) 62 The Gonococcal Arthritis-Dermatitis-Tenosynovitis

Syndrome 63 Foreign-Body Synovitis 64 Fungal Arthritis 66 Parasitic Arthritis 67 Syphilis and Other Diseases Caused by Flexibacteria 67 Viral Arthritis 68

Rubella: The "Catcher's Crouch" Syndrome 69 Hepatitis 69

Chronic Active Liver Disease (CALD) 70 Arthritis Associated with Other Infectious Agents 70 Arthropod-borne Arthritis 70

Rocky Mountain Spotted Fever 70 Lyme Disease 71

Special Syndromes Related to Infection 74 Musculoskeletal Manifestations of Subacute Bacterial

Endocarditis 74 Shunt Arthritis 74 Acne Rheumatism 74 Arthritis and Rash after Intestinal Bypass Surgery 75 Whipple's Disease 75 The Phalangeal Microgeodic Syndrome 75

Inflammatory Disorders 76 Inflammatory Bowel Disease (IBD) with Arthritis 76 Familial Mediterranean Fever (FMF) 79 Solitary Mastocytoma with Flushing 83

Contents IX

Relapsing Polychondritis 84 Idiopathic Periosteal Hyperostosis (Goldbloom's Syndrome) 89 Acute Pancreatitis with Arthritis 90 Pseudotumor of the Orbit 92 Cortical Hyperostoses 92

Caffey's Disease (Infantile Cortical Hyperostosis) 92 Melorheostosis 93

Sarcoid Arthritis 93 Pseudorheumatoid Nodules 94

Hypertrophic Osteoarthropathy 96 Secondary Hypertrophic Osteoarthropathy 96'

Cystic Fibrosis 97 Pseudoarthritis Associated with Congenital Absence of the

Intrahepatic Bile Ducts 97 Malignant Disease in Childhood Presenting as Musculoskeletal

Pain 98 Primary Hypertrophic Osteoarthropathy 98

Pachydermoperiostosis 100 Familial HO without Pachyderma 101

Unusual Traumas and Unusual Responses to Trauma 101 Acute Chondrolysis of the Hip (Juvenile Laminaire Coxitis) 101 Transitory Demineralization of the Hips 102 Child Abuse 104 Congenital Indifference to Pain (Charcot's Joints in

Childhood) 106 Frostbite 106 Stress Fractures 108 Foreign-Body Synovitis 108

Degenerative Disorders 108 Avascular ("Aseptic") Necrosis of Bone (AVN) 108 Cracking Joints 110 Chondromalacia Patellae 111 Osteochondritis Dissecans 111 Slipped Capital Femoral Epiphysis 111 Osteoarthritis in Childhood 112

Metabolic Diseases 113 Diabetic Cheiroarthropathy (The Diabetic Hand

Syndrome) 113 Idiopathic Juvenile Osteoporosis 116 Hyperparathyroidism 116 Late-Onset Rickets (and Tumor-Induced Rickets) 118 Endocrinopathies with Arthritis 118 Hypothyroidism 119 Hyperthyroidism 119 Cushing's Syndrome and Addison's Disease 120

X Contents

Vitamin and Fluoride Poisoning 120 Familial H yperli poproteinemia 122 Gout 122 Hyperuricemia and Secondary Gout 124 Alcaptonuria 124 Hemochromatosis 125 Kashin-Beck Disease (Urov Disease) 125 Calcific Periarthritis 126

Abnormal Blood Proteins 126 Hemoglobinopathies-Sickle-Cell Disease 126 Hemophilias and Thrombasthenias 127 Immunologic Deficiency Disorders with Arthritis 128 Familial Lipochrome Histiocytosis 129 Arthritis, Pyoderma Gangrenosum, and Streaking Leukocyte Factor 129 Waldenstrom's Hypergammaglobulinemic Purpura 132 Hereditary Angiodema (HAE) 133

Heritable Disorders Associated with Joint Laxity 133 Marfan's Syndrome 133 Ehlers-Danlos Syndrome (EDS) 134 Benign Hypermobility 135 Larsen's Syndrome 137

Joint Contractures at Birth 137 Nail-Patella Syndrome (Onycho-osteo-arthro Dysplasia) 137 Arthrogryposis Multiplex Congenita 138 Fetal Alcohol Syndrome 138 Congenital Contractual Arachnodactyly 138 Chromosomal Abnormalities 138 Punctate Epiphyseal Dysplasia 139 Congenital Joint Contractu res with Facial Abnormalities 139 The Stiff-Skin Syndromes 139

Hypomobile (Stiff-Joint) Syndromes Appearing after Birth 140 The Mucopolysaccharidoses and Mucolipidoses 144 The Weill-Marchesani Syndrome 144 Leri's Pleonosteosis 144 Myositis Ossificans (Fibrodysplasia Ossificans Progress iva) 144 Stickler Syndrome 144 Histiocytic Dermatoarthritis (Multicentric Reticulohistiocytosis) 145 The Lipidoses 145

Gaucher's Disease 145 Fabry's Disease 145 Ceramidosis (Farber's Disease) 146

Bone and Cartilage Dysplasias 147 Trichorhinophalangeal Dysplasia 147 Multiple Cartilaginous Exostoses 148 The Vanishing Bone Diseases 149 Winchester Syndrome 150

· Contents Xl

Congenital Arthropathy with Rash Appearing at Birth 151 Familial Hypertrophic Synovitis 151

Neoplasms and Neoplasm-like Lesions of the Bones and Joints 155 Tumors of Bone 155 Osteoid Osteoma 155 Benign Neoplasms of the Joints 158 Malignant Synovial Tumors 162

Psychiatric Disorders 162 Is the Disease "Organic" or "Functional"? 162 Psychogenic Rheumatism 164 Reflex Sympathetic Dystrophy 165 Munchausens' Syndrome and Factitious Fever 167

3 Juvenile Rheumatoid Arthritis 179 Diagnostic Criteria 179 Subtypes of JRA 179 Laboratory Studies 184 Radiographic Studies 185 Epidemiology of Arthritis in Childhood 188 Systemic JRA (Still's Disease) 188 Polyarticular Juvenile Rheumatoid Arthritis 214 Pauciarticular Juvenile Rheumatoid Arthritis 246 Iridocyclitis (Anterior Uveitis) 248 Arthritis with Psoriasis 253 Exercises as Therapy in Childhood Arthritis 254 Surgery in JRA 258

4 HLA-B27 -Associated Spondyloarthritis and Enthesopathy 274

Historical Considerations 276 The Histocompatibility System and Disease 279 HLA-B27 and Arthritis 281

Ankylosing Spondylitis 281 Reiter's Syndrome and "Reactive Arthritis" 282

HLA-B27 -Associated Spondyloarthritis and Enthesopathy 284 The Clinical Presentation of Children with B27 -Associated

Arthritis 285 Course of the Disease 300 Treatment 308

5 Systemic Lupus Erythematosus 313 Prologue 313 Epidemiology and Susceptibility 314 Immune Complexes and the Pathophysiology of SLE 319

Xli Contents

Clinical Measurement of Serum Immune Complexes, Complement, and Anti-DNA Antibodies 320

Tests for Antinuclear Antibodies and the Diagnosis of SLE 322 Renal Pathology in SLE 325 Es.timating Prognosis in SLE 332 Causes of Death in SLE 333 Spontaneous Remission in SLE 334 The Clinical Picture of SLE in Childhood 334

Presenting Manifestations 334 Diagnosis 335

Clinical Manifestations 336 SLE and Pregnancy 363 Lupus in the Neonate Drug Induced Lupus 365 Mixed Connective Tissue Disease 367 Discoid LE 369 Treatment of SLE in Children 370 Goals of Therapy 371 Principles of Therapy 371 Management Techniques 374

6 Dermatomyositis Clinical Presentation 390 Immunopathology 391 Epidemiology 392 Clinical Features Course of the Disease

392 403

Management and Prognosis 405 Disorders Resembling Dermatomyositis 406

Transient Acute Viral Myositis 406 Trichinosis and Hypereosinophilic Syndromes 407 Steroid Myopathy 407 Persistent and Fatal Central Nervous System Echovirus Infections in

Patients with Agammaglobulinemia 407 Pitfalls in Interpreting Serum CPK Activity 410 Hypothyroidism 410 Rhabdomyolysis and Lipid Storage Myopathy 411 Dermatomysitis and Malignancy 411 Muscular Dystrophy 411 Toxoplasmosis 411 Drug-Induced Dermatomyositis 411 Granulomatous Myositis 412 Chronic Congenital Myopathy Associated with Coxsackie

Virus A9 412

390

... Contents Xlll

7 Systemic Vasculitis Syndromes 415 Clinical Presentation 415

Kawasaki Disease 418 Polyarteritis Nodosa 442 Allergic Granulomatosis 446 Granulomatous Angiitis of the Brain 447 Steroid-Responsive Encephalomyelitis (Allergic Angiitis of the

Brain) 449 Takayasu's Arteritis 449 Familial Granulomatous Arteritis 453 Wegener's Granulomatosis 453 Limited Wegener's Granulomatosis 456 Lymphomatoid Granulomatosis 456 Hypersensitivity Vasculitis 458 Cutaneous Vasculitis 464 Beh~et's Syndrome 466 Cogan's Syndrome 469 Malignant Atrophic Papulosis (Kohlmeier-Degos Disease) 469 Juvenile Temporal Arteritis 470

Disorders Simulating Systemic Vasculitis 470 Left Atrial Myxoma 470 Hereditary Disorders of Amino Acid Metabolism Simulating

Systemic Vasculitis 471 Pheochromocytoma 472 Heritable Deficiency of Antithrombin III 473 Moyamoya 473 Hemiplegic Migraine 473 Goodpasture's Syndrome 473 Toxic Shock Syndrome 474

8 Scleroderma 484 Clinical Presentation 484 Immunopathology 487 Diagnosis 488 Epidemiology 489 Classification 491 Clinical Features 492 Course of the Disease 496 Treatment 497

Scleredema (The China-Doll Syndrome) 501 Subcutaneous Lipogranulomatosis (Rothmann-Makai

Syndrome) 502 Eosinophilic Fasciitis 504 Iatrogenic Fibrous Myopathy 507

XlV Contents

Drug-Chemical-Induced Scleroderma-like Disease 508 Scleroderma-like Syndrome as Part of Chronic Cutaneous Graft-

Versus-Host Reaction 508 Scleroderma Syndromes as Part of Human Adjuvant

Disease 508

9 Models of Connective Tissue Disease Experimentally Induced and Naturally Occurring Animal Models of

SLE 512 Animal Models of Virus-Induced Systemic Vasculitis-

Immune-Complex Vasculitis with Chronic Viremia 516 Vasculitis without Immune Complexes with Active Virus

in the Blood Vessel Walls . 516 Animal Models of Chronic Arthritis-Naturally Occurring

Chronic Infectious Arthritis of Animals 517 Chronic Arthritis Persisting after the Injection

of Infectious Materials 517 Arthritis Produced by the Remote Injection

of Noninfectious Antigens 518 Arthritis Produced by the Injection of Noninfectious

Material into Joints 518 Experimental Models of Autoimmune Muscle Disease 519 Experimental Models of Scleroderma 519 Tissue-Culture Models 519 Animal Models of Joint Mechanics 520

10 The Power of Positive Thinking Coping with Chronic Illness 524 Helping the Family Cope with Sickness 527 The Importance of Motor Activity in the Personality Development of

Children 529 The Importance of Physician Attitudes in the Care of the Chronically

III 529 Society's Attitudes toward the Crippled 531 School and the Crippled Child 532 Sex and the Disabled 534 Psychosocial Concepts in the Etiology of RA 534 Psychotherapy in the Care of Chronically III Children 535 Group Psychotherapy for Sick Children and Their Parents 536 Vocational Education for the Handicapped 537 Staying in the Mainstream 538

Index

511

522

542

Series Foreword

Comprehensive ManualS' in Pediatrics are designed to broaden the practitioner's clinical scope by providing a wide range of diagnostic and management skills ordi­narily considered to be the exclusive domain of the specialists. Although the series as a whole constitutes a comprehensive text in pediatrics, each volume stands on its own as a self-contained reference for the busy practitioner.

In order to maintain a uniform style and coverage of each subject, each manual is usually written by no more than one or two authors. Each author is an acknowl­edged expert in his or her field and provides a comprehensive, up-to-date account of the topic under discussion. Practically oriented, each volume offers concise guidelines and courses of treatment.

Michael Katz

E. Richard Stiehm

Foreword

Subspecialization in internal medicine and pediatrics is a relatively recent phe­nomenon. When Dr. Jerry C. Jacobs began to develop his special interest in rheu­matic disease approximately 25 years ago, there was a handful of pediatricians working in the field, and rheumatology was only slightly less strange than phre­nology as a term for a discipline of modern medicine. For better or worse, rheu­matology is an established subspecialty. Textbooks have been designed for the internist/rheumatol<lgist and monographs on the topic of chronic polyarthritis in children have been published. There are several reasons why a comprehensive rheumatology text for pediatricians is needed. Many of the important rheumatic syndromes in children are infrequently encountered in adults, e .. g., Kawasaki dis­ease, acute hematogenous osteomyelitis, acute chondrolysis of the hip, foreign­body synovitis, diabetic hand dystrophy, some patterns of juvenile chronic arthritis, etc., and the most common rheumatic diseases of adults rarely occur in pediatric age subjects. More important, the general approach to patients and their family, the techniques for elicitation of history and performance of physical examination, and the diagnostic skills termed (by the author) "pattern recognition" are different in adult and pediatric medicine. Chapter 2, "The Differential Diagnosis of Arthritis in Childhood," together with the chapter on "Clinical Techniques in Pediatric Rheumatology" would constitute a monograph of sort, worthy of the attention of pediatricians of any age or orientation.

Dr. Jacobs' respect for the basic as well as the creative side of medicine is evi­dent: "the importance of creative analytic listening by a physician who is intrigued and challenged by the problem and uses reason and imagination in his effort to solve it cannot be overestimated."

In Greek, Dr. Jacobs notes, the root for the words pain and punishment is the same. He summarizes some realities: "painful sickness is a discouraging business for patients and their families, interfering with developing independence and self­esteem and creating anxiety, fear and anger. Pessimism and gloom lead to increas-

XVlll Foreword

ing defeat and humiliation." In the final chapter, "The Power of Positive Think­ing," he presents a sensitive and pragmatic approach to such problems: "Not all symptoms are relieved by drugs. Healing is psychological, spiritual, emotional and social too."

There are a few things in the text that can be challenged. Many would disagree that "Rheumatology has been largely a stepchild of medicine, devoid of great dis­coveries." Not everyone shares Dr. Jacobs' views regarding the response to ther­apy and the prognosis of children with SLE nephritis or his interpretation of the HLA-B27 positive spondyloarthropathies, but these points are relatively minor and few in number, considering his contention (not disputed) that rheumatologists "thrive" on controversy and "rarely advocate an identical plan of therapy for a given patient."

In the Preface, the author indicates that the target audience for his text will consist largely of primary care pediatricians, but there are messages for all profes­sionals involved in the care of children with rheumatic disease, not only as a ref­erence source but as a statement of how an experienced and perceptive pediatric rheumatologist thinks and acts.

Charles L. Christian, M.D.

Physician in Chief Hospital for Special Surgery

New York, New York

Preface

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 multi authored 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 largely on personal experience, and where

*Paraphrased from Eric Bywaters, at the VIIIth European Congress of Rheumatology, Helsinki, Finland, June 1, 1975. See Bywaters EGL: The historical evolution of the concept of connective tissue disease. Scandinavian Journal of Rheumatology. Supplement 12:11-29,1976.

xx Preface

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 of monographs 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 out­standing sources of rheumatologic information on pages xxv-xxvi.

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 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

Preface XXI

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 what 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. Pi rani 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 Massini took some of the recent photographs. John W. Karapelou drew all of the wonderful figures.

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

I would like to thank, in particular, Larry Carter for his continuous encour­agement, which was badly needed, and Jerry L. Stone and William Gabello of Springer-Verlag. I am grateful to all of these individuals and to many others for their help and for their friendship.

Sources of Rheumatologic Information

Major Textbooks

Kelly WN, Harris ED Jr, Ruddy S, Sledge CB (eds.): Textbook of Rheumatology. W. B. Saunders, Philadelphia, 1981.

McCarty DJ (ed.): Arthritis and Allied Conditions, 9th edition. Lea and Febiger, Phil­adelphia, 1979.

Scott JT (ed.): Copeman's Textbook of the Rheumatic Diseases, 5th edition. Churchill Livingstone, New York, 1978.

Specialized Monographs

SLE

Dubois EL: Lupus Erythematosus, 2nd edition. University of Southern California Press, Los Angeles, 1974.

Fries JR, Holman HR: Systemic Lupus Erythematosus: A Clinical Analysis. Major Problems in Internal Medicine. W. B. Saunders, Philadelphia, 1975.

JRA

Brewer EJ Jr, Giannini EA, Person DA: Juvenile Rheumatoid Arthritis: Major Prob­lems in Clinical Pediatrics, Vol. VI, 2nd edition. W. B. Saunders, Philadelphia, 1982.

Miller JJ (ed.): Juvenile Rheumatoid Arthritis. PSG Publishing Co., Littleton, Massa­chusetts, 1979.

Schaller JG, Hanson V (eds.): Proceedings of the First ARA Conference on the Rheu­matic Diseases of Childhood. (Supplement to Arthritis and Rheumatism, 1977.) American Rheumatism Association.

XXVI Sources of Rheumatologic Information

Fink CW (ed.): Arthritis in Childhood. Report of the Eightieth Ross Conference on Pedi­atric Research. Ross Laboratories, 1981.

Arden GP, Ansell BM (eds.): Surgical Management of Juvenile Chronic Arthritis. Aca­demic Press, New York, 1978.

Spondyloarthritis

Wright V, Moll JMH: Seronegative Polyarthritis. Elsevier North-Holland, New York and Amsterdam, 1976.

Moll JMH (ed.): Ankylosing Spondylitis. Churchill Livingstone, New York, 1980. Calin A: Ankylosing Spondylitis. Medical Examination Publishing Co., Garden City,

New York, 1978. Scott, SG: A Monograph on Adolescent Spondylitis or Ankylosing Spondylitis: The Early

Diagnosis and Its Treatment by Wide-Field X-Ray Irradiation. Oxford University Press, Oxford, England, 1942.

Journals

Arthritis and Rheumatism This journal includes the Rheumatism Review, a summary of current literature, which is published every few years; it also includes annually, in the June issue, abstracts of papers presented at the annual meeting of the American Rheumatism Association. The journal also publishes supplements on special subjects in rheumatology.

Annals of the Rheumatic Diseases Journal of Rheumatology Scandinavian Journal of Rheumatology Clinics in the Rheumatic Diseases Bulletin on the Rheumatic Diseases Seminars in Arthritis and Rheumatism