Pediatric rheumatology is one of the newer specific areas defined in the field of pediatrics. Most of the conditions encompassed in this category are not new to the active pediatric practitioner. Almost all of us, I am sure, have at times diagnosed and treated children with rheumatoid arthritis, and occasionally we meet with cases of lupus erythematosus. Less frequently seen are children with polyarteritis, dermatomyositis, scleroderma, Schönlein-Henoch purpura, and various other forms of arteritis and vasculitis.
The present symposium on rheumatology deals with the most recent advances and diagnoses in this field. It is under the Guest Editorship of Dr. Jerry C. Jacobs, Professor of Clinical Pediatrics and Director of the Section of Pediatric Rheumatology at the College of Physicians and Surgeons of Columbia University in New York City. Dr. Jacobs is recognized among the foremost pediatric rheumatologists in the United States.
In this review are included two new disease entities - Kawasaki disease and Lyme disease. I've always been bothered by the concept of "new" diseases. Are these really new diseases with new etiologies and symptom complexes, or were our clinical and laboratory abilities inadequate to diagnose diseases that were already prevalent?
Consider, for instance, Kawasaki disease, a pathological condition with very specific and unusual symptomatology. Here is a syndrome with fever, rash, conjunctivitis, strawberry tongue, acute pharyngitis, and cervical adenopathy. There is also joint pain and swelling and gastrointestinal symptoms. So far it reminds me of scarlatina. But many of the children develop arthritis with aneurysms of the coronary artery and peripheral artery as well.
Kawasaki first described the condition in Japan in 1961. The Japanese University of Health inaugurated a Kawasaki disease survey in 1970, and in 1985 (after their eighth survey) the cases reported and followed totaled 63,000. Was this essentially a Japanese disease, much like race-limited diseases such as Mediterranean anemia or sickle cell anemia? But then the disease, once the diagnostic picture was clear, began making its appearance in the United States. By 1980, Moreus et al, at the Centers for Disease Control in Atlanta, reported on 445 cases nationally, a large number of which were white children.
It is still hard for me to believe that a disease of such magnitude never existed before. I spoke to Dr. Jacobs of my disbelief, and he mentioned to me that in 1896 the first recognizable case was reported in the Journal of St. Bartholomews Hospital in England. It was entitled, "Scarlatinal Dropsy with Infantile Polyarthritis and Aneurysm."
Anyway, it still remains a mystery disease for me. It is still a disease of unknown etiology, there is no evidence of person-to-person transmission, laboratory studies have been nonproductive, and so far genetic disposition has not been identified. Also, why, in many instances, do the aneurysms subside spontaneously?
Rheumatology is an interesting field, and for many of die diseases much more must be learned about etiology and treatment. In this present symposium, Dr. Jacobs has aimed at bringing us up to date in new and important areas of this field.
The first article is on the "Use of Nonsteroidal Anti-Inflammatory Drugs in Children." It was contributed by Dr. Earl J. Brewer, Clinical Professor of Pediatrics and Head of the Pediatric Rheumatology Section of the Baylor College of Medicine; and Dr. Ivonne Arroyo, Pediatric Rheumatology Fellow at the same institution. This is an important and valuable article. Most of us in treating rheumatoid arthritis have resorted to the use of aspirin, but if this was ineffective, switched to limited periods of prednisone.
Dr. Brewer, with a wealth of experience in the use of the new chemotherapeutic anti-inflammatory agents, describes the usage of three groups of nonsteroidal drugs that are anti-inflammatory, analgesic, and antiseptic. As with aspirin, all of these drugs are rapid-acting for relief of pain and fever, but may take somewhat longer for their antiinflammatory effect.
The authors note that although none of the nonsteroid drugs is superior in action to aspirin, aspirin tends to produce more adverse reactions. At the present time, only one of these drugs, tolmetin sodium (Tolectin), has been approved by the FDA for use with children. The others will probably be approved in the near future. This article should be of great value in the treatment of rheumatoid arthritis and related diseases.
The second article discusses "Lyme Disease," another of the recently diagnosed diseases. This paper is authored by Dr. Andrew H. Eichenfield of the Pediatric Rheumatology Center of the Children's Seashore House and the Children's Hospital of Philadelphia. He is also Assistant Professor of Pediatrics at the University of Pennsylvania School of Medicine.
This is a clear description and discussion of the disease, first recognized in this country in 1975, with its symptom complex of rash, then neurological signs, asymmetrical arthritis and at times cardiac signs. This is one of those medical detective stories - with the eventual offender identified as the deer tick. This tick transmits a spirochete responsible for the disease.
Dr. Eichenfield carefully presents the clinical manifestations of the disease, the laboratory features and serology, and finally the most recent treatment. He notes that by means of serologic testing one can, in endemic areas, differentiate juvenile rheumatoid arthritis from Lyme disease where a rash has not been present.
The third contribution to the symposium deals with the "Diagnosis and Management of Systemic Lupus Erythematosus in Childhood. " It has been written by Dr. Ilona S. Szer of the Department of Pediatric Rheumatology at the Floating Hospital for Infants and Children, New England Medical Center. Dr. Szer is also Assistant Professor of Pediatrics at the Tufts University School of Medicine, Boston, Massachusetts.
This is a very complex disease with numerous affected organs. With this knowledge, each case must be individualized. The disease, with its many manifestations, is being diagnosed with increasing frequency during the period of childhood and adolescence. In my own experience, the malar rash has been the first presenting sign, followed by lymphadenopathy and polyarthritis. Dr. Szer takes us through the numerous clinical manifestations of this disease and the most effective treatment at the present time.
Systemic lupus erythematosus is a disease that the alert pediatrician will diagnose with increasing frequency and must treat with great medical understanding. It is a serious disease which can be controlled with proper treatment. The next article continues a discussion of lupus erythematosus but focuses on the neonatal period. It has been written by Dr. Rosemarie Watson of the Department of Dermatology of the Johns Hopkins Medical College.
The syndrome of neonatal lupus erythematosus has been receiving increasing attention in recent years. Clinically, the newborns may have a lupus rash and/or a congenital heart block. But the telltale finding is the presence of Ro (SSA) antibody in both mother and infant.
Dr. Watson brings us a full account of this syndrome where 50% of the infants have complete heart block which is permanent. The rash usually subsides within 6 months and the antibodies disappear with the resolution of the rash.
In this article, suggested therapy for prenatally detected heart block or cardiomyopathy is presented. As far as prognosis is concerned, it is stated that in the absence of complete heart block the prognosis is excellent. It is interesting to note that in this lupus syndrome arthritis does not appear.
The final paper is by Dr. Jerry Jacobs, the Guest Editor of this symposium. It discusses the "Management Strategies in Kawasaki Disease." Dr. Jacobs has been at the forefront of the study of this disease for the past 10 years, and especially in the area of therapy. The effort must be made to prevent the formation of aneurysms and thrombi.
In 1977, Dr. Jacobs reported on the successful treatment of Kawasaki disease with high-dose aspirin. In this present article, this mention of the successful treatment of Kawasaki disease with this method of therapy is further amplified, emphasizing the need for early treatment of the disease for adequate control. However, the author warns against using high-dose aspirin without daily monitoring of salicylate levels.
Dr. Jacobs mentions other methods of therapy now being used, such as steroids and gamma globulin in Japanese studies. An important part of this paper deals with treatment during the subacute phase of the disease and the follow-up procedures. In conclusion, it is noted that 88% to 94% of children with Kawasaki disease recover completely.
The articles in this symposium are most interesting and present information not to be found in any of the recent pediatric textbooks.