Biomedical subjects
Publications and source records attributed to Rebecca ten Cate.
Treatment of systemic vasculitides is usually based on the use of corticosteroids and other immunosuppressive drugs. We describe a 10-year-old girl with systemic vasculitis resistant to immunosuppressive treatment who had a rapid and impressive response to treatment with infliximab.
OBJECTIVE: To evaluate the sensitivity to change of a newly developed radiologic assessment tool, the Dijkstra score, and to develop a numeric composite score and progressor classification scheme to apply in juvenile idiopathic arthritis (JIA) trials. METHODS: A placebo-controlled trial of sulfasalazine (SSZ) in patients with oligoarticular- and polyarticular-onset JIA yielded the data for this study. Data were obtained from 418 sets of radiographs of the clinically involved and contralateral joints (at study entry and at 6 months' followup) from 66 JIA patients. The Dijkstra score assesses the presence or absence of swelling, osteopenia, joint space narrowing, growth abnormalities, subchondral bone cysts, erosions, and malalignment. These signs were combined in the Dijkstra composite score, to assess inflammation (DI), growth (DG), and damage (DD). Progression was defined as an increase in either the DG or the DD score. Scores were evaluated among all radiographs, a standard set of films (hand, foot, and knee), and per patient. All scores were used to explore differences between the 2 treatment groups. RESULTS: Over time, 58% of joints remained normal, 23% remained abnormal but stable, 14% showed an increase in signs, and 5% showed a decrease in signs. Of the 66 JIA patients, 12% had normal radiographic findings throughout followup, 27% showed abnormalities at some sites without change, and 61% showed change in at least 1 site. Changes in the DI, DG, and DD scores varied considerably per type of joint and occurred most frequently in joints of the standard set. DI and DG scores changed most often in the knees, while DD scores changed primarily in the hands and feet. The disease course in 8% of joints was classified as progressive. Films of SSZ-treated patients, versus the placebo group, showed less deterioration by the DD scores (P = 0.04), and the disease course was more often classified as nonprogressive in the SSZ group (P = 0.037). When progressors were defined as those who had at least one radiograph showing progression, significantly more placebo-treated patients were considered progressors (P = 0.046). CONCLUSION: In this trial data set, the Dijkstra composite score and the resulting progressor classification system are comprehensive and feasible tools that are sensitive to change and discriminate between clinical situations. They should now be tested by other investigators and in other data sets.
OBJECTIVE: To study occurrence as well as clinical signs and symptoms of temporomandibular joint (TMJ) involvement in juvenile idiopathic arthritis (JIA) in a population representing all subtypes of JIA. METHODS: Ninety-seven consecutive children with JIA underwent orthodontic evaluation including an orthopantomogram (OPG). Further evaluation included patient characteristics, disease onset, course, and medical treatment. RESULTS: Forty-five percent of all children had TMJ involvement. Frequencies according to JIA subtypes: systemic 67%, oligoarticular (persistent and extended) 39%, rheumatoid factor (RF) negative polyarticular 59%, RF positive polyarticular 33%, enthesitis related arthritis 13%, psoriatic arthritis 33%, and other arthritis 50%. In children with a polyarticular course, irrespective of their disease onset, TMJ involvement was more frequent (55% vs 31% in oligoarticular course). In children with disease onset at a young age and/or an extended course of the disease, TMJ involvement was also more frequent. Pain during jaw excursion, absence of translation, asymmetry during maximal opening and protrusion, as well as crepitation during evaluation are predictors for TMJ involvement with a good specificity but a low sensitivity. Not all patients with TMJ involvement have clinical signs. CONCLUSION: Because of the high prevalence and discrepancy between clinical signs and presence of arthritis of the TMJ, regular orthodontic evaluation and OPG is recommended to recognize TMJ involvement and enable early intervention.
UNLABELLED: Two young boys had a severe uveitis which was difficult to treat. They both developed psoriasis located in the pubic area almost 1 year and several years later respectively. One boy also developed arthritis of an intermittent character. Both boys were ANA and HLA-B27 negative. CONCLUSION: in children, the possible association between uveitis and psoriasis with or without arthritis has to be kept in mind.
OBJECTIVE: To describe radiologic features of patients with juvenile idiopathic arthritis (JIA) in a standardized manner, to test the reliability and feasibility of this description, and to correlate these features with clinical signs as a first step in the development of a standardized assessment method. METHODS: The placebo-controlled study of sulfasalazine in patients with oligoarticular, extended oligoarticular, and polyarticular JIA performed by the Dutch Juvenile Idiopathic Arthritis Study Group yielded the data for this study. All trial entry radiographs (clinically involved joints and contralateral joints) were scored (in consensus by a skeletal radiologist and pediatric rheumatologist) for the presence of swelling, osteopenia, joint space narrowing, growth abnormalities, subchondral bone cysts, erosions, and malalignment. RESULTS: Data on 67 of 69 patients were analyzed. The mean age was 9.1 years (range 2.5-17.6 years), and the median disease duration was 24 months (range 5-176 months). Thirteen percent of the patients were IgM rheumatoid factor (IgM-RF) positive, and 16% were HLA-B27 positive. All 68 clinically evaluated joints were included in the maximum of 19 radiographed joints (or joint groups) per patient. The mean number of radiographed joints per patient was 7 (range 2-15); knees, hands, ankles, and feet were most frequently affected. Fifty-eight patients (87%) had radiologic abnormalities in at least one joint (soft-tissue swelling in 63% of patients, growth disturbances in 48%, joint space narrowing in 28%, and erosions in 15%). In total, half of the radiographs of the clinically involved joints showed radiologic abnormalities, including two-thirds of the radiographs of the clinically affected hands and knees. Univariate analysis revealed a good correlation between the overall articular (clinical) severity and the presence of radiologic abnormalities (odds ratio [OR] 1.38, P < 0.0001). Multivariate analysis showed increased ORs for the presence of radiologic abnormalities and IgM-RF positivity (OR 4.6, P = 0.005) or HLA-B27 positivity (OR 3.0, P = 0.004). In general, reproducibility of the radiologic scoring method was good (mean kappa coefficient of 0.74 [range 0.40-0.86]), although there were scoring discrepancies for swelling, osteopenia, and growth disturbances. The scoring took 10-20 minutes per patient. CONCLUSION: Our model of describing and scoring radiologic abnormalities of radiographed joints in JIA was feasible, mostly reproducible, correlated well with the overall articular severity score, and added substantial new information not available on clinical examination.
OBJECTIVE: To determine if anti-cyclic citrullinated peptide antibodies (anti-CCP) can be detected in sera of patients with juvenile idiopathic arthritis (JIA) and if they can be used to identify patients with a more destructive course of disease. METHODS: One hundred serum samples of 71 patients with JIA taken at different time points in their disease course were analyzed by a commercially available anti-CCP ELISA. Followup serum samples from 28 patients were also tested. Correlations between anti-CCP and disease characteristics, medication, and radiological damage (presence of joint space narrowing and/or erosions) were also determined. RESULTS: The serum samples came from patients of all 8 different subtypes of JIA (mean age: 9.6 years, median: 10.5; disease duration mean: 39 months, median: 24) including 11 polyarticular rheumatoid factor positive (IgM-RF) patients. Anti-CCP was positive in 73% of the IgM-RF positive JIA patients and in 3% of the other JIA patients (p < 0.0001). Disease duration, medication, and anti-nuclear antibody positivity did not differ significantly between anti-CCP positive and negative patients. Testing of followup samples showed almost identical anti-CCP results. All IgM-RF positive JIA patients had radiological damage (p < 0.001). Of the anti-CCP positive patients, 80% had radiological damage resulting in a significant difference between anti-CCP positive and negative patients (p = 0.009) with an odds ratio (OR) of 12.7, but corrected for IgM-RF, the OR was no longer significant (p = 0.88). CONCLUSION: Anti-CCP antibodies can be detected in the sera of patients with JIA but almost exclusively in the subset of patients with polyarticular IgM-RF.
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