Depression and medication nonadherence in childhood-onset systemic lupus erythematosus.

Objectives Depression and medication nonadherence are important in managing chronic diseases, but little is known about these factors in childhood-onset systemic lupus erythematosus (cSLE). The objectives of this cross-sectional study were to estimate prevalence of depression and medication nonadherence, describe demographic and disease characteristics associated with depression and medication nonadherence, and evaluate the association between depression and medication nonadherence in cSLE patients. Methods Patients with cSLE ( n = 51) completed validated screening questionnaires to identify depression and medication nonadherence, Patient Health Questionnaire-9 and Medication Adherence Self-Report Inventory, respectively. Demographic and disease characteristics were obtained via chart abstraction, and compared between groups of depression or medication nonadherence status. A multivariable linear regression model adjusting for propensity scores was conducted to evaluate the association between depression and medication nonadherence. Results The prevalence of a positive depression screen was 58.8%, and seven patients reported suicidal ideation (13.7%). The prevalence of self-reported medication nonadherence was 19.7%. No statistically significant differences for demographic and disease characteristics were found between patients with a positive vs. negative depression screen. Patients reporting medication nonadherence were more likely to have longer disease duration (4.8 vs. 2.6 years, p = 0.035). As the severity of depression symptoms increased, the degree of medication nonadherence also increased (beta = -1.89; p = 0.011). Conclusions The prevalence of depression and medication nonadherence is high in cSLE, and these factors have a direct relationship. Interventions that better recognize and treat depression and increase rates of medication adherence are needed to improve outcomes in cSLE.

Disease outcomes and ovarian function of childhood-onset systemic lupus erythematosus.

The objective of this study was to determine the medical outcomes including the ovarian function childhood-onset SLE (cSLE). The medical records of all patients diagnosed with cSLE in the Greater Cincinnati area between 1981 and 2002 were reviewed. Patient interviews were performed to obtain additional information on current medication regimens, disease activity [SLE Disease Activity Index (SLEDAI-2k)], and damage [Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SDI)]. The occurence of premature ovarian failure (POF) and reduction of the ovarian reserve was assessed by timed gonadotropin levels. There were 77 patients (F : M = 70 : 7, 53% Caucasian, 45% African-American and 2% Asian) with a mean age at diagnosis of 14.6 years. Nine patients died (88.3% survival) during the mean follow-up of 7.1 years (standard deviation [SD] 5.6) and 88% of the patients continued to have active disease (SLEDAI-2k mean/SD: 6.6/6.7), with 42% of them having disease damage (SDI mean/SD: 1.62/2.1); Non-Caucasian patients had higher disease activity (mean SLEDAI-2k: 10 versus 3.4; P < 0.0001) and more disease damage (mean SDI : 2.1 versus 1.2; P < 0.02) than Caucasian patients. Cyclophosphamide was given to 47% of the patients during the course of their disease and associated with the presence of significantly reduced ovarian reserve (RR = 2.8; 95% CI: 1.7-4.8; P = 0.026). Patient mortality and disease damage with cSLE continue to be high. Although overt POF with cyclophosphamide exposure is rare, it is a risk factor for significantly decreased ovarian reserve cSLE.

Knee in early juvenile rheumatoid arthritis: MR imaging findings.

PURPOSE: To determine the magnetic resonance (MR) imaging findings in the knee in early juvenile rheumatoid arthritis. MATERIALS AND METHODS: MR imaging (1.5 T) was performed in the more symptomatic knee in 30 children with juvenile rheumatoid arthritis with a symptom duration 1 year or less. Conventional, fast spin-echo, three-dimensional gradient-echo, and gadolinium-enhanced T1-weighted images were assessed. Two radiologists independently read the images, and a third resolved disagreements. These images were compared with knee radiographs in 27 children. RESULTS: Mean maximal synovial thickness was 4.8 mm +/- 2.4 (SD). Mean synovial volume was 15.4 mL +/- 10.8. Suprapatellar joint effusions were seen in 26 (87%) of 30 knees, meniscal hypoplasia in 11 (37%) of 30 knees, and abnormal epiphyseal marrow in eight (27%) of 30 knees. Three knees had articular cartilage contour irregularity, fissures, and/or thinning. One knee had a bone erosion. Knee radiographs showed suprapatellar fullness in 78% of the knees, joint space narrowing in one knee, and no bone abnormalities. CONCLUSION: Synovial hypertrophy and joint effusions are the most frequent MR imaging findings of knees in early juvenile rheumatoid arthritis. Early in the disease, radiographically occult cartilage and bone erosions are uncommonly seen at MR imaging. The potential relationship of synovitis to cartilage abnormalities deserves further study.

Chemokine receptor CCR4 on CD4+ T cells in juvenile rheumatoid arthritis synovial fluid defines a subset of cells with increased IL-4:IFN-gamma mRNA ratios.

To understand the mechanisms that promote recruitment and survival of T cells within the pediatric inflamed joint, we have studied the expression of CCR4 and CCR5 on synovial fluid T cells and matched peripheral blood samples from juvenile rheumatoid arthritis (JRA) patients using three-color flow cytometric analysis. Thymus- and activation-regulated chemokine and macrophage-derived chemokine, ligands for CCR4, were measured by ELISA in JRA synovial fluid, JRA plasma, adult rheumatoid arthritis synovial fluid, and normal plasma. IL-4 and IFN-gamma mRNA production was assessed in CD4+/CCR4+ and CD4+/CCR4(-) cell subsets. We found accumulations of both CCR4+ and CCR5+ T cells in JRA synovial fluids and a correlation for increased numbers of CCR4+ T cells in samples collected early in the disease process. Thymus- and activation-regulated chemokine was detected in JRA synovial fluid and plasma samples, but not in adult rheumatoid arthritis synovial fluid or control plasma. Macrophage-derived chemokine was present in all samples. CD4+/CCR4+ synovial lymphocytes produced more IL-4 and less IFN-gamma than CD4+/CCR4(-) cells. These findings suggest that CCR4+ T cells in the JRA joint may function early in disease in an anti-inflammatory capacity through the production of type 2 cytokines and may play a role in determining disease phenotype.

Outcome in pediatric rheumatic disease.

Outcome assessment has been a focus of recent research in rheumatic diseases and in pediatric rheumatology. The establishment of a preliminary core set of outcome variables for juvenile rheumatoid arthritis patients and a preliminary definition of improvement using these variables have been important steps toward standardization of outcome assessment. A population-based epidemiologic study has added to our knowledge of long-term outcome in juvenile arthritis and confirms the chronicity of disease that was found in previous studies. Standardization of outcome assessment in pediatric systemic lupus erythematosus has not been achieved to the same extent as in juvenile rheumatoid arthritis, but renal survival and overall mortality are important outcomes in this disease that are easily quantified. Recent studies of pediatric systemic lupus erythematosus demonstrate similarities with respect to organ involvement and overall survival between adult and pediatric lupus patients.

Population dynamics of the deer mouse (Peromyscus maniculatus) and Sin Nombre virus, California Channel Islands.

Hantavirus pulmonary syndrome, first documented in 1993, is caused by Sin Nombre virus (SNV), which is carried by the Peromyscus species. In 1994, high SNV antibody prevalence was identified in deer mice from two California Channel Islands. We sampled two locations on three islands to estimate mouse population density and SNV prevalence. Population flux and SNV prevalence appear to vary independently.

Magnetic resonance imaging in juvenile rheumatoid arthritis.

OBJECTIVES: This study was undertaken to examine the role of magnetic resonance imaging (MRI) in the management of patients with juvenile rheumatoid arthritis (JRA). METHODS: A Medline search was performed to identify all publications pertaining to MRI in JRA. Further searches and identification of articles from bibliographies identified articles pertaining to plain radiograph imaging in JRA and MRI in adult rheumatoid arthritis. RESULTS: Studies in both children and adults have shown superiority of MRI to plain radiography in detecting synovial hypertrophy and articular cartilage abnormalities. Several studies in adults have shown a good correlation of MR images with gross anatomic as well as histological findings at arthroscopy. Biochemical changes that occur before structural changes within the joint may even be detectable with magnetic resonance spectroscopy. CONCLUSIONS: MRI holds promise for the assessment of disease progression in JRA and may be useful in serial evaluation, as in clinical trials.

Juvenile rheumatoid arthritis--assessment.

The assessment of overall health status of a child with juvenile rheumatoid arthritis (JRA) is complex and multi-dimensional. The general physical examination is complemented by a rheumatological evaluation that includes determination of articular indices of inflammation and duration of inactivity stiffness. Laboratory assessment plays a critical role in monitoring side effects of pharmacologic management, but is limited in its ability to portray accurately the degree of active inflammation. Newly measureable indicators of inflammatory activity, such as serum cytokine and soluble cytokine receptors will likely become part of routine laboratory assessment in the future. Radiographs remain a useful tool for assessing disease progression, but may be replaced in the future by magnetic resonance imaging. In recent years, rheumatologists have realized that measurement of overall physical and psycho-social functional ability, quality of life, and pain are major descriptors to consider during routine follow-up. They are also critical in the assessment of long-term clinical effectiveness. The importance of nutritional assessment has also been realized. This section seeks to describe some of the methodologic approaches currently used to assess the variables mentioned above, and includes a brief discussion of the evolving instrumentation which attempts to measure variables of a more cognitive or subjective aspect.

Regional myocardial infarction of low-flow type in a neonate with tricuspid atresia.

Clinical and anatomical data are presented on a neonate with tricuspid atresia, who died at 16 hours with a regional, nontransmural myocardial infarcti after clinical documentation of akinesis. A partially occlusive intimal lesion associated with acute angulation of the right coronary artery was noted in an area of fibrosis that included the atretic tricuspid valve. Low-flow myocardial infarction was found in the posterior part of the left ventricle in an area supplied by the right coronary artery distal to the arterial lesion.