Antinuclear antibodies and lupus-like syndromes in children receiving anticonvulsants.

Drug-induced systemic lupus erythematosus (SLE)-like syndromes in children are most commonly associated with the administration of ethosuximide, diphenylhydantoin, and trimethadione. Five children receiving ethosuximide who presented with syndromes suggestive of SLE were studied. Each and fever, malar rash, arthritis, and lymphadenopathy. Two children had pleural effusions and another developed myocarditis and pericarditis. Three patients had anti-DNA antibodies associated with low serum C3. In four of five children symptoms disappeared with the discontinuation of ethosuximide; two of these continue to have antinuclear antibodies (ANA). One child continues to have active SLE with nephritis. A group of 101 children from a seizure clinic were tested for the presence of ANA. ANA were found in 14 of 70 children receiving ethosuximide and/or diphenylhydantoin; 2 of 14 had anti-DNA antibodies. Serum ANA titers in the drug-induced SLE group did not differ significantly from those of the asymptomatic seizure patients. ANA were also present in 5 of 23 children receiving phenobarbital only. The induction of ANA by phenobarbital is a possible hypothesis. Quantitative immunoglobulins and C3 were not significantly altered in the asymptomatic children with ANA. Follow-up studies at ten months showed no asymptomatic child with ANA to have developed clinical with ANA to have developed clinical evidence of SLE. This study suggests that asymptomatic children who develop ANA should have careful observation, but need not have their anticonvulsants discontinued.

Use of western blot and enzyme-linked immunosorbent assays to assist in the diagnosis of Lyme disease.

Without evidence of erythema chronicum migrans, diagnostic confirmation of Lyme disease may be difficult, particularly if there are conflicting laboratory results. Often, for families and physicians, the clinical dilemma is whether fatigue, arthritis/arthralgias, a positive enzyme-linked immunosorbent assay (ELISA), and tick exposure, but no evidence of erythema chronicum migrans, are sufficient to diagnose and treat Lyme disease. Patients with discordant ELISA and Western blot (WB) assay results for Borrelia burgdorferi were studied to determine whether there was sufficient clinical evidence to support a diagnosis of Lyme disease. Of 650 consecutive sera analyzed by ELISA in a laboratory within a 1-year period, 77 were subsequently tested by WB. The clinical data from these patients were then analyzed. The study population was divided into three groups: group 1 (positive ELISA, positive WB), group 2 (positive ELISA, negative WB), and group 3 (negative ELISA, negative WB). Findings included the following: (1) Patients with a strong clinical history of Lyme disease were usually positive by both WB and ELISA (group 1). (2) All patients with erythema chronicum migrans had both positive WB and ELISA tests. (3) Ninety-one percent of group 2 had a rheumatic or inflammatory condition other than Lyme disease. (4) A definite response to antibiotics occurred in 75% of patients wherein both ELISA and WB were positive but in only 11% of cases with a positive ELISA but a negative WB. (5) History of tick exposure and degree of fever were not significantly different among the three serologic groups, and thus they were not diagnostically helpful.(ABSTRACT TRUNCATED AT 250 WORDS)

Sarcoidosis and systemic vasculitis.

BACKGROUND: Systemic vasculitis is an unusual complication of sarcoidosis. Over a 10-year period, the authors have provided care for six patients who had features of both sarcoidosis and vasculitis. Vasculitis could not be attributed to other causes. OBJECTIVES: To report six patients (five children) who had sarcoidosis and systemic vasculitis and compare our experience with previous literature. To better delineate the clinical spectrum of sarcoid vasculitis and its response to therapy. METHODS: Retrospective analysis and a Medline literature review of sarcoid and concurrent vasculitis from 1966. RESULTS: Our six patients had systemic illnesses that included fever, peripheral adenopathy, hilar adenopathy, rash, pulmonary parenchymal disease, musculoskeletal symptoms, and scleritis or iridocyclitis. Biopsies revealed features compatible with the diagnosis of sarcoidosis or necrotizing sarcoid granulomata in either skin, lymph node, lung, synovium, bone, bone marrow, liver, trachea, or sclera. Arteriography showed features of large vessel vasculitis in three patients, all of whom were African American, whereas patients with small vessel vasculitis were white. Prior reports of sarcoid and vasculitis included 14 adults, of whom half had predominantly small vessel disease, and half had medium- or large-sized vessel disease. Eight previously reported children included seven with primarily large vessel sarcoid vasculitis. Racial background was noted in 15 reported cases and included whites (6), African Americans (5), and Asians (4). Among the authors' six patients, four improved when treated with prednisone alone. However, relapses occurred when the drug was tapered or withdrawn. CONCLUSIONS: Sarcoidosis may be complicated by systemic vasculitis that can affect small- to large-caliber vessels. Sarcoid vasculitis can mimic hypersensitivity vasculitis, polyarteritis nodosa, microscopic polyangiitis, or Takayasu's arteritis. African American and Asian patients are disproportionately represented among cases with large vessel involvement. Corticosteroid and cytotoxic therapy is palliative for all forms of sarcoid vasculitis. However, relapses and morbidity from disease and treatment is common.

Rheumatic diseases of childhood.

Development of diagnostic criteria for juvenile rheumatoid arthritis, systemic lupus erythematosus, a juvenile dermatomyositis, as well as advances in molecular biology, have assisted epidemiologic study of the rheumatic disorders of childhood. It may be misleading to extrapolate the incidence and prevalence of pediatric forms of arthritis from population studies of adults. Additional study of the frequency of childhood musculoskeletal disorders is very much needed. Classification criteria for Kawasaki syndrome, fibrositis in children, and the juvenile spondyloarthropathies are also desirable.

Future directions for pediatric rheumatology as an academic specialty. Survey of pediatric department chairpersons.

A survey of pediatric department chairpersons examines the growth and future of pediatric rheumatology training programs. Chairpersons are queried about certain departmental characteristics, in order to determine if any of these correlate with attitudes about pediatric rheumatology.

Emergency and critical care issues in pediatric rheumatology.

The study of critical care issues in pediatric rheumatology is in its infancy, and the available literature is largely case studies and small retrospective series. A child's limited communication skills and the lack of understanding of disease consequences by both parent and child may cause both overuse and underuse of emergency services. To the extent that small case experiences in children with rheumatic diseases do not adequately reflect possible disease presentations or diagnostic and treatment options, pediatric and adult rheumatologists, intensivists, and emergency physicians should readily turn as needed to larger reported experiences in adults with similar conditions.

Methotrexate in the treatment of juvenile rheumatoid arthritis and other pediatric rheumatoid and nonrheumatic disorders.

The goal of treatment for juvenile rheumatoid arthritis (JRA) and other pediatric rheumatic disorders is to minimize joint destruction, pain, and deformity and to maximize all aspects of growth and development. Oral and injectable methotrexate are now often given early in the treatment of JRA, childhood dermatomyositis, difficult-to-control arthritis in the pediatric spondyloarthropathies, SLE, sarcoidosis, several of the vasculopathies, and idiopathic iritis. Weekly low-dose MTX has become a mainstay of long-term improved control of these disorders, and is associated with strikingly few documented long-term side effects. Dosages, pharmacology, side effects, efficacy, and treatment strategies are discussed. Although formal studies are lacking, MTX for the pediatric rheumatic disorders seems to be associated with less frequent physician visits, lower total costs, improved function, and fewer late reconstructive surgeries.

Health status (arthritis impact) in children with chronic rheumatic diseases. Current measurement issues and an approach to instrument design.

Arthritis outcome measures in adults have evolved from biologic "disease activity," to observations of motor/movement based "functional status," to assessment of "health status." This monograph suggests a framework for developing a pediatric arthritis health status (impact) instrument in which measurable global outcome dimensions include psychological, social, ADL, pain, school, family, behavioral, and developmental variables. Physical and laboratory parameters of inflammation/disease activity are not part of either functional status or arthritis impact, but rather perhaps the most important predictor variable of outcome. Juvenile rheumatoid arthritis (JRA) is a prototypic chronic disease for development of a health status instrument for children. Prior obstacles have been (a) that systemic, polyarticular, and pauciarticular JRA appear to have very different amounts of impact on health status; and (b) although juvenile arthritis has proven a useful term for medical and public education, its value as a study entry criterion for functional status instrument development is limited. Any new pediatric arthritis impact instrument must fulfill five criteria: enable quantification; demonstrate reliability, validity, and precision; and specify data collection procedures. Important design issues include (a) developing a self-report format for children, (b) ensuring generalizability across age groups, and (c) the parent as proxy in most patient-related dialogue and interventions. Instrument elements could come from 8-10 adult and pediatric health/functional status instruments already in use. By 10 years of age, children can provide much independent information. Whether global assessments by patient, parent, and physician should be included, or be a separate "gold standard," is not yet clear. The creation of better health status measures is an important and formidable challenge for pediatric rheumatology.

Reflex sympathetic dystrophy in children. A physical therapy approach.

Children with reflex sympathetic dystrophy (RSD) almost always receive physical therapy as part of a multidisciplinary approach, but there is controversy about the efficacy of many alternative modalities. In a retrospective chart review of 24 females and 12 males with 49 episodes of RSD (mean age at onset, 13.4 years), the average time to correct diagnosis was 9.4 months (median, 4.2 months; range, 1-53 months). Sixteen ankles, 12 knees, eight wrists, two hips, and two shoulders were involved. Psychological assessments revealed significant abnormalities in 25 (83%) of 30 children evaluated. Thirty-four (94%) of 36 children received physical therapy including a wide variety of nonstandardized approaches. Children with one to two episodes of RSD averaged 4.0 physical therapy modalities; unresolved cases had 8.9 modalities attempted. Time from the first RSD episode to resolution averaged 9.0 months in 69% of children. Incorrect diagnoses prolonged many initial episodes; following correct diagnosis, symptom resolution occurred in 3.1 months. Recurrences are common, and 25% of children still exhibited RSD symptoms at last follow-up.

Physical fitness levels in children with polyarticular juvenile rheumatoid arthritis.

Children with juvenile rheumatoid arthritis (JRA) often exhibit fatigue and prolonged exercise recovery. Improved fitness through physical conditioning has not been a goal of standard medical or physical treatment regimens for JRA, and fitness levels of children with JRA have rarely been studied. We compared physical fitness in 20 6 to 11-year-old patients with polyarticular JRA with sex-, age-, and size-matched controls, using the Health Related Physical Fitness Test (HRPFT), a national, standardized, norm-referenced test. We correlated fitness scores with summary joint counts, and with an articular severity index (sum of joint swelling, tenderness, pain, and limited range for each child). The results showed that children with polyarticular JRA were less physically fit than normally active (noncompetitively athletic) children of the same sex, age, and size. There was no statistically significant relationship between increased joint counts, and/or disease severity scores, and reduced fitness scores. This suggests that physical fitness levels are less related to degree of "disease activity" than is often thought. We conclude that (1) a readily available, nationally standardized fitness test can be used to assess children with JRA: and (2) fitness levels and measures of disease activity do not correlate. We believe that multiple factors, perhaps including family, physician, and school concerns about potential disease exacerbation following exercise, may account for the low fitness levels observed in children with JRA.

Scleroderma in childhood.

Scleroderma is a rare disease in children. Heterogeneous in its many forms, it ranges from circumscribed and self-limited pigmentary disorders to disabling and disfiguring involvement of an extremity. It also may include diffuse skin disease, multiple internal organ derangements, and a rapidly fatal outcome. Outcome varies widely and is related to the location and extent of cutaneous involvement, evaluation of disfigurement or impairment, and which treatment resources are available.

Physical fitness in children with juvenile rheumatoid arthritis and other chronic pediatric illnesses.

This article discusses the importance of physical fitness and exercise in healthy adults and children, and differentiates the terms "physical fitness" and "physical activity". The benefits of physical activity for adults and children with chronic illnesses and rheumatic diseases are emphasized.

Orbital and childhood sarcoidosis.

Orbital infiltration, anterior uveitis, disc edema, choroiditis, erythema nodosum, and polyarticular arthritis due to sarcoidosis occurred in a five-year-old child who presented with unilateral proptosis. Diagnosis was enhanced by computerized tomography of the orbit, and confirmed by synovial biopsy findings. This patient's course illustrates the importance of thorough and frequent ocular examinations in childhood uveitis associated with systemic disease and demonstrates an unusual finding of orbital sarcoidosis. Childhood sarcoid arthritis may be misdiagnosed as juvenile rheumatoid arthritis. Familiarity with the ocular and systemic findings may lead to the correct diagnosis.

Reflex sympathetic dystrophy in children: an orthopedic perspective.

To design diagnostic criteria for reflex sympathetic dystrophy (RSD) and to initiate a prospective treatment protocol, we reviewed our experience with 49 episodes of RSD in 36 children. There were 24 females and 12 males; mean age at diagnosis was 13.4 years (range: 8 to 19); mean time from pain onset to correct diagnosis was 9.2 months (range: 1 to 53). Lower extremity involvement predominated. Pain was "severe" in 61%, and skin color changes, swelling, hyperesthesia, abnormal skin temperatures, muscle weakness, and decreased range of motion were all present in at least 75% of cases. Osteopenia was observed in 15 of 38 radiographs; of 24 bone scans, 7 were normal, 11 showed increased uptake, and 6 demonstrated decreased uptake. Of the 23 children who had psychological evaluations, 83% revealed some type of significant emotional dysfunction. Analgesic and antiinflammatory medications were not helpful, nor were local injections or regional blockades effective. An inpatient diagnostic and rehabilitation program for treating chronic pain, including orthopedics, rheumatology, psychology, and twice-daily physical therapy was most likely to lead to resumption of age-appropriate activities. Despite extensive physiological testing, physician, parent, and/or patient reluctance to accept absence of a primary organic disease was common. We present diagnostic criteria for pediatric RSD.

Quality assurance and continuous quality improvement: history, current practice, and future directions.

Quality Assurance (QA) provides opportunities for physicians and allied health professionals to improve patient care and disease outcomes. Its goals are increased efficiency and efficacy in healthcare. QA activities are based upon objective criteria and systematic review and make important contributions to the effectiveness of hospitals and other care facilities. Successful programs help to maximize health status of patients while minimizing resource utilization. Beginning in 1917, early QA efforts were often informal and subjective but now include standards for QA and strategies for monitoring and evaluating patient care. Central to its new "Agenda for Change," the Joint Commission on Accreditation of Healthcare Organizations has embraced the concept of continuous quality improvement (CQI). This moves the focus of review away from department- or practitioner-specific activities and toward a "systems" form of evaluation. CQI is rooted in patient-care realities, is easy to implement, is based upon scientific assessments, and solves practical problems in an incremental and ongoing fashion.

Congenital complete heart block and SSA antibodies: obstetric implications.

Mothers with known or occult rheumatic disorders may be delivered of infants with congenital complete heart block. The more frequent use of ultrasonography during pregnancy now allows early detection of heart block in utero. The transplacental passage of SSA or SSB antibodies, of the IgG class, may mediate or be associated with immune damage to the fetal cardiac conduction system, as reported in our two patients. Maternal and/or newborn screening for SSA and SSB antibodies in selected patients permits an early presumptive diagnosis and will assist perinatal planning, particularly for immediate newborn cardiac pacemaker implantation. Early serologic detection of such antibodies may also assist family counseling of mothers at risk and should promote investigation of techniques to modify the immune status of these mothers. SSA- or SSB-positive maternal/fetal pairs should be prospectively managed by the obstetrician, neonatologist, and rheumatologist.