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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 However, relapses occurred when the drug was tapered or withdrawn.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.

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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.

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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.

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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.

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OBJECTIVE: To investigate the potential clinical utility of serial levels of sIL2-R as a marker of disease activity among children with juvenile rheumatoid arthritis (JRA) treated with methotrexate (MTX). METHODS: sIL2-R levels, measured by ELISA, were evaluated in 16 JRA patients (10 polyarticular, six systemic-onset) treated with oral, weekly MTX. sIL2-R values were compared with those of 49 normal controls. Medical record review was used to obtain relevant clinical data. Joint counts (number of swollen joints) were used as indicators of clinical change. A reduction of 50% in joint counts between pre and post treatment measurements was considered a clinically significant response. RESULTS: The mean (SEM) sIL2-R value of pre treatment JRA of 1728(290) U/ml was significantly higher than the post treatment value of 921(229) U/ml (Wilcoxon Rank test, p < or = 0.001). Pre treatment values were also significantly different from the mean(SEM) of healthy controls of 519(19) U/ml (p < 0.001). Pre treatment sIL2-R levels of 2417(291) U/ml in systemic-onset JRA were significantly higher than sIL2-R values in polyarticular JRA patients of 1218(884) U/ml (Mann-Whitney rank test p < 0.001). Among the 13/16 children with good therapeutic responses (> or = 50% improved), the range of sIL2-R decreases was 154-2641 U/ml (mean 842 U/ml); sIL2-R levels increased in the three children with poor clinical responses to methotrexate. CONCLUSIONS: sIL2-R levels paralleled the course of disease in all patients. sIL2-R levels may be useful for monitoring therapeutic responses in children with JRA.

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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.

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There are few observations of inflammatory synovitis in association with specific chromosomal abnormalities in children or adults. We review the genetic and rheumatic disease literature and describe the clinical, radiologic and pathologic features of a 14-year-old boy with trisomy 5q, terminal 2p deletion, developmental delay, and a 5-year course of a polyarticular, symmetrical arthropathy similar to juvenile rheumatoid arthritis. He was treated with multiple nonsteroidal antiinflammatory drugs, intramuscular gold, and oral methotrexate, but developed iridocyclitis, joint space narrowing with erosions, and multiple flexion contractures; disease progression slowed after addition of chlorambucil. The frequency and manner of association of genetic disorders with inflammatory arthropathies is presently unknown. Additionally, children with 2 major disabilities often require aggressive medical intervention to maximize their potential for adult independence.

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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.

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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.

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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)

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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.

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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.

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Twenty-nine children with juvenile rheumatoid arthritis were studied to determine the safety and efficacy of methotrexate therapy. The initial dose of methotrexate averaged 7.1 mg/m2/wk and was given as a single, oral weekly dose or as three divided doses, each separated by 12 hours. Current antiinflammatory medications were continued; 25 of 29 children had had lack of efficacy, and 8 of 29 had toxic effects, with one or more prior drugs such as intramuscularly or orally administered gold, hydroxychloroquine, or D-penicillamine. Intolerable corticosteroid dependency or toxic effects were present in 18 of 29 cases. Methotrexate-treated patients were examined monthly; minimum treatment duration required to assess efficacy and toxicity was 6 months. The range of treatment duration was 8 to 39 months (mean 18.5 months). Efficacy was assessed by comparing pretreatment versus posttreatment fever and rash, swollen-joint counts, articular indexes, duration of morning stiffness, functional class, hemoglobin levels, and platelet counts. Treatment with methotrexate effectively controlled fever and rash in 83% of children with systemic juvenile rheumatoid arthritis, reduced morning stiffness by 63%, eliminated recalcitrant joint restriction in 48%, and reduced numbers of swollen joints and swelling indexes by 46% and 52%, respectively. No significant toxic effects were observed. Juvenile rheumatoid arthritis of long duration, or with major erosions, was more likely to be refractory to methotrexate therapy. We recommend earlier consideration of methotrexate in place of other slow-acting antirheumatic drugs for juvenile rheumatoid arthritis not responding well to usual therapy. Future studies should address potential methotrexate toxic effects in the lungs and reproductive system, as well as outcome after discontinuation of methotrexate treatment.

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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.

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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.

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