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OBJECTIVE: To compare the clinical, functional, and radiographic outcomes in patients with enthesitis-related arthritis (ERA) with those in patients with other subtypes of juvenile idiopathic arthritis (JIA) and healthy controls, and to determine genetic markers, patient characteristics, and early disease variables that predict the development of remission, sacroiliitis, and physical limitations in ERA. METHODS: Fifty-five children with ERA who were first admitted to Rikshospitalet Medical Center between 1980 and 1985 were studied. Patients with oligoarthritis or polyarthritis who were admitted during the same period (n = 55) and individuals from a national population registry (n = 55) were matched for sex and age and used as controls. Health status was assessed after a median of 15.3 years of disease (range 11.7-21.9 years) and, in some patients, was reassessed after a median of 23.0 years (range 19.7-29.4 years) of disease, by use of the 36-item Short Form health survey and the Health Assessment Questionnaire. Clinical and radiographic examinations were performed at the 15-year followup visit. Variables relating to the onset of disease were retrospectively obtained by chart review. HLA alleles were determined by genotyping and serologic testing. RESULTS: Patients with ERA had lower levels of physical functioning, poorer physical health, and more bodily pain compared with patients with oligoarthritis or polyarthritis (after a median of 15.3 and a median of 23.0 years) and normal controls (after a median of 15.3 years). Among patients with ERA, remission occurred in 44%, sacroiliitis was observed in 35%, and reduced spinal flexion was observed in 75%. Predictors of failure to attain disease remission included the following: ankylosing spondylitis (AS) in a first-degree relative, the presence of HLA-DRB1*08, and ankle arthritis within the first 6 months. HLA-DPB1*02 was a protective factor, whereas a persistently elevated erythrocyte sedimentation rate (ESR), and hip arthritis within the first 6 months were risk factors for sacroiliitis. Female sex, a family history of AS, and high numbers of affected joints within the first 6 months predicted poor physical health status after 23 years. Male sex was associated with reduced anterior flexion of the spine. CONCLUSION: In this study, patients with ERA had poorer physical outcomes compared with patients with oligoarticular or polyarticular JIA and controls from the general population. A family history of related diseases, sex, the presence of HLA-DRB1*08, the absence of HLA-DPB1*02, a persistently elevated ESR, early hip or ankle arthritis, and high numbers of affected joints were predictors of an unfavorable outcome. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To determine the frequency of osteopenia in patients with childhood-onset systemic lupus erythematosus (SLE) compared with that in healthy matched controls, and to evaluate the relationship between disease-related variables and bone mineral mass. METHODS: Bone mineral density (BMD) and bone mineral content (BMC) were measured in a cohort of 70 patients with childhood-onset SLE (mean +/- SD disease duration 10.8 +/- 8.3 years, mean +/- SD age 26.4 +/- 9.9 years) and 70 age- and sex-matched healthy controls. BMD and BMC of the femoral neck, lumbar spine, total body, and distal one-third of the radius were measured by dual x-ray absorptiometry. We investigated the relationship between BMC and the following disease variables: cumulative dose of corticosteroids, organ damage, current use of corticosteroids, use of cyclophosphamide, age at disease onset, and disease activity at the time of diagnosis. Biochemical markers of bone metabolism were also measured. RESULTS: BMD values for the lumbar spine and femoral neck were significantly lower in patients than in healthy controls. The reduction in BMD of the lumbar spine was significantly greater than that of the total body. In multiple linear regression analyses, a higher cumulative corticosteroid dose was significantly associated with lower BMC of the lumbar spine and femoral neck. Decreased lumbar spine BMC was also related to male sex. CONCLUSION: The frequency of osteopenia was higher in patients with childhood-onset SLE than in matched controls. The lumbar spine was the most seriously affected skeletal site, followed by the femoral neck. The cumulative dose of corticosteroids was shown to be an important explanatory variable for BMC values in the lumbar spine and femoral neck. [ABSTRACT FROM AUTHOR]

OBJECTIVE: To explore early changes and predictors of bone mass in children with juvenile idiopathic arthritis (JIA) in order to identify patients who will develop bone mass reductions. METHODS: We conducted a prospective cohort study of 108 children with early JIA (ages 6-18 years; mean disease duration 19.3 months) who were individually matched with 108 healthy children for age, sex, race, and county of residence. Bone mass and changes in total body, spine, femur, and forearm bone mineral density and bone mineral content (BMC), body composition, growth, and biochemical parameters of bone turnover were examined at baseline and at followup a mean of 24 months later. Low bone mass was defined as a Z score >1 SD below the reference population. RESULTS: Of the 200 children evaluated at followup, the 100 healthy children had greater gains in total body BMC (P = 0.035), distal radius BMC (P < 0.001), and total body lean mass (P < 0.001) than did the 100 JIA patients. Low or very low total body BMC was observed in 24% of the patients and 12% of the healthy children. Bone formation, bone resorption, and weight-bearing activities were reduced in the patients compared with the healthy children. Multiple regression analysis showed that in patients with JIA, serum bone-specific alkaline phosphatase, serum C-telopeptide of type I collagen, and weight-bearing activities were independent predictors of changes in total body BMC. Total body BMC was lower in patients with polyarticular onset than in those with oligoarticular disease onset. CONCLUSION: Patients with JIA have moderate reductions in bone mass gains, bone turnover, and total body lean mass early in the disease course. [ABSTRACT FROM AUTHOR]

Objective: To determine the frequency of low bone mineral content (BMC) and low bone mineral density (BMD) as long-term complications in adolescents with early-onset juvenile idiopathic arthritis (JIA), and to identify disease variables, patient characteristics, and biochemical bone markers related to low bone mass., Methods: One hundred five (87%) of 121 adolescent patients with early-onset JIA (ages 13-19 years, 80 girls and 25 boys, mean age at onset of JIA 2.8 years), from a cohort first admitted to the hospital between 1980 and 1985, were assessed after a mean disease duration of 14.2 years. BMC and BMD of the total body, the lumbar spine at L2-L4, and the femoral neck were measured by dual-energy x-ray absorptiometry. Age- and sex-specific reference values from a pooled, healthy reference population were used to calculate Z scores. Low bone mass was defined as a Z score less than -1 SD., Results: Among the 103 adolescent JIA patients who underwent total-body imaging, 41% had low total-body BMC and 34% had low total-body BMD. Compared with adolescent JIA patients who had normal total-body BMC, those with low BMC had lower mean weight (P < 0.001), height (P < 0.001), lean mass (P < 0.001), and remission rates (P = 0.016), had longer duration of active disease (P = 0.013), had higher numbers of active and mobility-restricted joints (P < 0.001 and P = 0.001, respectively), had more disability (P = 0.011), had higher frequencies of joint erosions (P < 0.001), and had higher erythrocyte sedimentation rates (P = 0.033). In multiple linear regression analyses of total-body BMC, 88% of the variance was explained by the duration of active disease, the number of joints with restricted mobility, the bone area, urinary deoxypyridinoline values, age, weight, and height., Conclusion: Forty-one percent of the adolescents with early-onset JIA had low bone mass >11 years after disease onset. The development of low total-body BMC was related to the duration of active disease, disease severity, measures of bone resorption, weight, and height.

Objective: To assess the frequency of sacroiliitis and the radiographic and clinical outcome in juvenile idiopathic arthritis (JIA) and determine patient characteristics, early disease variables, and genetic markers that predict development of sacroiliitis., Methods: We performed a retrospective cohort study of 314 (79%) of the 400 JIA patients first admitted to the hospital between 1980 and 1985. The participants were examined after a median disease duration of 14.9 years (range 11.7-25.1). Radiographs of the sacroiliac joints, hips, ankles, and tarsi were obtained and studied in a blinded manner by 2 radiologists. The presence of HLA-DRB1 and DPB1 alleles was determined by genotyping and that of HLA-B27 by serologic testing. Variables relating to the onset and course of the disease were obtained by chart reviews., Results: Twenty (6%) of the JIA patients developed radiographic sacroiliitis according to the New York criteria. In 9 patients (45%), sacroiliitis had not been demonstrated before the followup examination. At followup, spinal flexion (lateral and anterior) was reduced in 70-75% of patients with sacroiliitis and in 30-35% of those without sacroiliitis. Compared with the JIA patients without sacroiliitis, those with sacroiliitis more frequently had inflammatory back pain, enthesitis, radiographic changes in the hips and calcanei, erosions of any peripheral joint, and uveitis. Predictors of sacroiliitis were HLA-B27, absence of DPB1*02, hip joint involvement within the first 6 months, and disease onset after age 8 years. The following factors were more common among patients in whom sacroiliitis developed than in other JIA patients: DRB1*04, male sex, family history of ankylosing spondylitis, psoriasis, inflammatory back pain, and enthesitis within the first 6 months., Conclusion: In the current study, radiographically evident sacroiliitis had developed in 6% of JIA patients after a median disease duration of 14.9 years. HLA-B27, absence of DPB1*02, late onset of disease, and early hip involvement were predictors of sacroiliitis.