Current medication choices in juvenile rheumatoid arthritis II--update of a survey performed in 1993.

The documentation of treatments used for Juvenile Rheumatoid Arthritis (JRA) is important to allow for the evaluation of practice patterns for future outcome studies. A survey of nine pediatric rheumatologists was performed between September 1999 and February 2000. Each of the physicians prospectively recorded demographic and treatment information on consecutively sampled JRA patients (n=395). Pauciarticular onset JRA was present in 46%, polyarticular onset JRA in 35%, and systemic onset JRA in 19% of the children. Naproxen was the most frequently prescribed medication (55% of the patients), followed by methotrexate (MTX), which was used in 39% of the patients. Folic acid supplementation (1 mg/day) was provided to 69% of the patients treated with MTX. Etanercept was used in 11% of the children. Eleven percent of the patients received corticosteroids, and 13% of children on corticosteroids took calcium supplements. Uveitis was present in 8% and had a chronic course in 79% of those cases. Although systemic medications were used in 50% of the children with uveitis to control eye inflammation, severe damage to the eyes developed in 30% of them. Fourteen percent of the patients required gastroprotective medications. Compared with findings of a similar survey performed in 1993, there was no significant change in the frequency of use of naproxen, but nabumetone is now more often prescribed, and COX-2 inhibitors have been introduced in the therapy of JRA. Changes among second-line agents used for JRA have also occurred, although there was no change in the frequency of use of MTX or corticosteroids. JRA continues to be a treatment challenge for the practicing pediatric rheumatologist. Patients often show incomplete response to the currently available medications. Therefore, new therapeutic agents need to be evaluated for their use in JRA, and the treatment of JRA associated uveitis especially needs to be improved.

Is it juvenile rheumatoid arthritis or fibromyalgia?

For the clinician evaluating adolescents with chronic musculoskeletal pain and fatigue, the distinctions between JRA and FS are clear based on physical examination findings. The two conditions can coexist. For the patient with an initial diagnosis of either JRA or FS whose clinical response to therapy is not in keeping with expectations or physical examination findings or whose clinical course worsens without explanation, reevaluation to determine if FS in the JRA patient has developed or JRA in the FS patient has emerged is warranted. Until clinicians have a better understanding of the intricacies of the neurohormonal and immunologic systems and how they affect somatic symptoms, they can continue to provide patients with a treatment plan based on current knowledge that should minimize patients' discomfort and allow them to have appropriately functional lives.

Development of validated disease activity and damage indices for the juvenile idiopathic inflammatory myopathies. II. The Childhood Myositis Assessment Scale (CMAS): a quantitative tool for the evaluation of muscle function. The Juvenile Dermatomyositis Disease Activity Collaborative Study Group.

OBJECTIVE: To develop, validate, and determine the measurement characteristics of a quantitative tool for assessing the severity of muscle involvement in children with idiopathic inflammatory myopathies. METHODS: The Childhood Myositis Assessment Scale (CMAS) was developed from 2 existing observational functional assessment tools to assess muscle function in the areas of strength and endurance across a wide range of ability and ages. The 14 ordinal items included were chosen to assess primarily axial and proximal muscle groups and are ranked with standard performance and scoring methods. Following the development of the CMAS, a training video and written instructions were developed and reviewed by the physicians participating in this study. Subsequently, utilizing a randomized block design, 12 physicians independently scored 10 children (9 with dermatomyositis, 1 with polymyositis; ages 4-15 years) twice in one day (morning and afternoon) on the CMAS. A pediatric physical therapist performed quantitative manual muscle strength testing (MMT) twice on each child (morning and afternoon), including the neck, trunk, and proximal and distal extremity muscle groups. RESULTS: The CMAS has a potential range of 0-51, with higher scores indicating greater muscle strength and endurance. The observed mean for the 10 patients was 36.4 (median 44, SD 14.1, observed range 5-51). The total score for the CMAS correlated with the physician's global assessment (by visual analog scale) of disease activity, the MMT score, serum creatine kinase level, and the Juvenile Arthritis Functional Assessment Report score. The score on the CMAS was not correlated with patient age. Interrater reliability (Kendall's coefficient of concordance) ranged from 0.77 to 1.0 for individual items (all P < 0.001), and overall, it was 0.95 (P < 0.001). Intrarater reliability for the individual physicians was measured by correlation of the CMAS scores for each patient on 2 separate evaluations and ranged from 0.97 to 0.99, with an overall correlation for all physicians of 0.98 (all P < 0.001). CONCLUSION: The CMAS demonstrated an acceptable range of observed scores, excellent convergent validity, and excellent inter- and intrarater reliability. The CMAS is validated to quantitatively assess muscle function in the areas of strength and endurance in children with idiopathic inflammatory myopathies. It can be used in routine clinical care as well as therapeutic trials.

Psychological symptoms in chronic fatigue and juvenile rheumatoid arthritis.

OBJECTIVE: To determine if psychological morbidity in youth with chronic fatigue is caused by the stress of coping with a chronic illness. STUDY DESIGN: Case-control study comparing pediatric patients with debilitating chronic fatigue and matched subjects with juvenile rheumatoid arthritis, a chronic medical illness with similar functional sequelae. SETTING: Pediatric Infectious Diseases Clinic and Juvenile Rheumatoid Arthritis Clinic of Kosair Children's Hospital. PARTICIPANTS: Nineteen children and adolescents with debilitating chronic fatigue and 19 age- and sex-matched peers with juvenile rheumatoid arthritis. Outcome. Structured Interview, Kaufman Brief Intelligence Test, Child Behavior Checklist, and Youth Self-Report. RESULTS: Intellectual functioning on the Kaufman Brief Intelligence Test Composite was average (103, standard score) for both groups. Pediatric patients with chronic fatigue had higher levels of internalizing psychological distress than patients suffering from juvenile rheumatoid arthritis, despite the fact that both groups had a similar pattern of decline in social and physical activities. Duration of illness did not explain the difference in psychological symptoms. CONCLUSIONS: Psychological factors may play a more active role in debilitating chronic fatigue in pediatric patients than can be explained by the stress of coping with a similar chronic, non-life-threatening illness.

Pulmonary manifestations of rheumatic diseases.

The pulmonary manifestations of rheumatic diseases can vary substantially, both in severity and mechanism of injury. There is still relatively little experience with these conditions in the pediatric age group. Perhaps tissue diagnosis will help elucidate the pathophysiology involved and allow for the development of more specific treatment protocols.

Medication choices in juvenile rheumatoid arthritis.

This survey was performed to review medication usage by pediatric rheumatologists in the care of patients with juvenile rheumatoid arthritis (JRA). Prospective data from 50 patients per physician with JRA were recorded by six pediatric rheumatologists in the Fall of 1993. Naproxen was used most frequently-in 48% of all patients. Next in order of frequency were methotrexate (39%), prednisone (15%), tolmetin (12%), indomethacin (11%) and folic acid (10%). Salicylates (acetylsalicylic acid, trisalicylate and salsalate) were used in 7%, and myochrysine was used in 2% of patients. Overall, nonsteroidal anti-inflammatory drugs were used in 93% of all patients, slower-acting antirheumatic drugs (SAARDs) were used in 54% and prednisone in 15%.Medication usage varied by disease type in predictable ways but also varied by physician in ways that could not be accounted for by population differences. Methotrexate was the most-often used of all SAARDs and supplanted myochrysine in JRA. Naproxen was the most often used NSAID in the treatment of JRA and had largely supplanted salicylates. With the arrival of practice guidelines, reasons for and impact of these changes (as well as the interesting variations between physicians) will need to be examined.

Expressive therapy in pediatric and adolescent gynecology.

Children and adolescents seen by gynecologists may have a broad spectrum of emotional needs that can affect the gynecologic examination as well as the young person's overall well-being. The field of expressive therapy is a relatively new one. The therapist uses a variety of techniques to allow the patient to explore feelings, attitudes, or events in ways that may help the patient to develop new strategies to cope with distress. The application of expressive therapy in pediatric and adolescent gynecology appears to be ever more encompassing.

Systemic lupus erythematosus: pulmonary presentation in childhood.

Pulmonary symptoms as the initial or primary manifestation of SLE are rare. When pulmonary symptoms are present, they occur most commonly when other organ systems are involved. The absence of skin and renal involvement, the presence of normal serum complement, and the poor response to corticosteroids in this patient are of interest. Pulmonary disease produced by childhood SLE may represent, as in adults, a subgroup of SLE disease. Our report emphasizes the importance of recognizing diffuse interstitial infiltrates as an initial symptom of childhood SLE even in the absence of more obvious signs.

Annular atrophic connective tissue panniculitis of the ankles.

A case of annular lipoatrophy of the ankles in a 4-year-old girl is presented. Histologically a lobular lymphohistiocytic panniculitis with masses of foam cells was present. This pattern resembles that seen in atrophic connective tissue panniculitis. The patient responded to treatment with prednisone and dapsone and now has residual lipoatrophy.

Endometriosis in association with uterine anomaly.

Endometriosis is frequently a chronic process, which may begin soon after menarche. The process may be enhanced by mechanical obstruction. Theories of retrograde menstruation and metaplasia still remain in vogue. Endometriosis is a cause of both acute and chronic pelvic pain in the adolescent. We present case reports of müllerian lateral wall fusion defects with surgical correction and evidence for resorption of endometriosis. Clinicians must be aware that patients with uterine anomalies may develop extensive endometriosis, which upon creation of an unobstructed outflow tract results in complete resorption. Furthermore, the mechanism of formation of endometriosis in association with an outflow tract obstruction may be very different from that associated with infertility. We recommend consideration of endometriosis and/or a reproductive tract abnormality in the adolescent with persistent pelvic pain.

Gonadal and müllerian duct agenesis in a girl with 46,X,i(Xq).

A unique case of gonadal agenesis and rudimentary müllerian duct development in association with a 46,X,i(Xq) karyotype is reported. The patient presented with short stature and subtle features of Turner syndrome. Endocrine evaluation revealed elevated gonadotropins and cytogenetic findings from both peripheral blood leukocytes and skin fibroblasts were consistent with a 46,X,i(Xq) karyotype. Laparoscopy revealed both uterus and gonads to be absent. Developmental failure of the müllerian system in association with gonadal agenesis in a patient with 46,X,i(Xq) has not been previously reported. The basis for müllerian duct regression in this patient remains unclear. Recommendations for treatment are made.

Solvent abuse associated pulmonary abnormalities.

Organic solvent inhalation leading to intoxication has become an important medical problem. Pulmonary function studies were performed on 42 young (ages 11-31) solvent inhalers and on 20 controls (ages 10-26). Ventilatory studies were normal in the majority, but residual volumes of inhalers were significantly higher (136.26% +/- 32.64% S.C.) than in controls (104% +/- 27.78% S.C.)--p less than 0.001. Lung tissue obtained at autopsy, from three known solvent inhalers was found to contain microscopic abnormalities similar to those seen in experimental panlobular emphysema and it is assumed these changes are consistent with increased residual volumes.

Solvent abuse associated cortical atrophy.

Eleven of 42 toluene inhalers were evaluated with computed tomography scans because of neurologic abnormalities. Six of the 11 were found to have cerebral cortical atrophy. In addition, two of the six had cerebellar atrophy. All six had been exposed to toluene for at least 10 years. This study suggests that atrophy of the central nervous system may occur in chronic toluene abusers.