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1.
Ocul Immunol Inflamm ; 30(6): 1384-1398, 2022 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-33826468

RESUMO

PURPOSE: To present a national guideline for ophthalmologic care and surveillance of juvenile idiopathic arthritis-associated uveitis (JIA-uveitis). METHODS: Review article based on medical literature and the experience of an Expert Committee composed of members of the Brazilian Society of Pediatric Ophthalmology/Brazilian Council of Ophthalmology and the Brazilian Society of Pediatrics/Brazilian Society of Rheumatology. Studies with a high level of evidence were selected by searching the PubMed/Medline database. The final document was approved by the experts. RESULTS: The main recommendations are that children/adolescents with JIA should undergo screening according to their risk factors. Ophthalmological checkups should also consider ocular inflammation and therapy. Topical glucocorticoids should be the first line of therapy, with systemic glucocorticoids acting as bridge treatments in severe uveitis. Methotrexate should be the first-line systemic therapy and anti-tumor necrosis factor (anti-TNF alpha) the second for uncontrolled uveitis. CONCLUSIONS: This evidence-based guideline for JIA-uveitis will be useful for both ophthalmology and rheumatology practice.


Assuntos
Artrite Juvenil , Uveíte , Adolescente , Criança , Humanos , Artrite Juvenil/complicações , Artrite Juvenil/diagnóstico , Artrite Juvenil/tratamento farmacológico , Inibidores do Fator de Necrose Tumoral , Brasil/epidemiologia , Uveíte/diagnóstico , Uveíte/tratamento farmacológico , Uveíte/etiologia , Glucocorticoides/uso terapêutico , Fator de Necrose Tumoral alfa , Prática Clínica Baseada em Evidências
2.
Scand J Rheumatol ; 43(4): 329-33, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-25087579

RESUMO

OBJECTIVES: Our aim was to demonstrate the benefit of whole-body magnetic resonance imaging (WBMRI) as a diagnostic modality in the detection of muscle activity in juvenile dermatomyositis (JDM)/polymyositis (JPM) patients and to correlate these findings with clinical evaluation, laboratory examinations, nailfold capillaroscopy (NFC), and muscle biopsy. METHOD: Thirty-four patients aged 5.5 to 18.9 years with a diagnosis of JDM/JPM were prospectively evaluated using clinical examination, muscle enzyme determination, the Childhood Myositis Assessment Scale (CMAS), Disease Activity Score (DAS), Manual Muscle Testing (MMT), NFC, and WBMRI. An open muscle biopsy was performed if muscle disease activity was detected on WBMRI. RESULTS: Disease activity was detected in WBMRI in four (11.7%) patients and confirmed by muscle biopsy. All four patients had elevation of at least one muscle enzyme and NFC showed scleroderma patterns in these patients. CONCLUSIONS: WBMRI allows us to evaluate the extent and symmetry of muscle disease and inflammatory activity. NFC is an important additional examination to assess disease activity.


Assuntos
Dermatomiosite/diagnóstico , Imageamento por Ressonância Magnética/métodos , Músculo Esquelético/patologia , Polimiosite/diagnóstico , Adolescente , Brasil , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Estudos Prospectivos , Índice de Gravidade de Doença
3.
Braz. j. med. biol. res ; 47(8): 721-726, 08/2014. tab
Artigo em Inglês | LILACS | ID: lil-716277

RESUMO

We evaluated the concentrations of 25-hydroxyvitamin D [25(OH)D] in children and adolescents with juvenile systemic lupus erythematosus (JSLE) and associated them with disease duration and activity, use of medication (chloroquine and glucocorticoids), vitamin D intake, calcium and alkaline phosphatase levels, and bone mineral density. Thirty patients with JSLE were evaluated and compared to 30 healthy individuals, who were age and gender matched. Assessment was performed of clinical status, disease activity, anthropometry, laboratory markers, and bone mineral density. The 30 patients included 25 (83.3%) females and 16 (53.3%) Caucasians, with a mean age of 13.7 years. The mean age at diagnosis was 10.5 years and mean disease duration was 3.4 years. Mean levels of calcium, albumin, and alkaline phosphatase were significantly lower in patients with JSLE compared with controls (P<0.001, P=0.006, and P<0.001, respectively). Twenty-nine patients (97%) and 23 controls (77%) had 25(OH)D concentrations lower than 32 ng/mL, with significant differences between them (P<0.001). Fifteen patients (50%) had vitamin D levels <20 ng/mL and 14 had vitamin D levels between 20 and 32 ng/mL. However, these values were not associated with greater disease activity, higher levels of parathormone, medication intake, or bone mineral density. Vitamin D concentrations were similar with regard to ethnic group, body mass index, height for age, and pubertal stage. Significantly more frequently than in controls, we observed insufficient serum concentrations of 25(OH)D in patients with JSLE; however, we did not observe any association with disease activity, higher levels of parathormone, lower levels of alkaline phosphatase, use of medications, or bone mineral density alterations.


Assuntos
Adolescente , Criança , Feminino , Humanos , Masculino , Adulto Jovem , Conservadores da Densidade Óssea/uso terapêutico , Lúpus Eritematoso Sistêmico/sangue , Vitamina D/análogos & derivados , Vitamina D/uso terapêutico , Fosfatase Alcalina/sangue , Antirreumáticos/uso terapêutico , Densidade Óssea , Estudos Transversais , Cálcio/sangue , Cloroquina/uso terapêutico , População Branca , Glucocorticoides/uso terapêutico , Medições Luminescentes , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Hormônio Paratireóideo/sangue , Estatísticas não Paramétricas , Albumina Sérica/análise , Vitamina D/sangue
4.
Braz J Med Biol Res ; 47(8): 721-6, 2014 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-25055165

RESUMO

We evaluated the concentrations of 25-hydroxyvitamin D [25(OH)D] in children and adolescents with juvenile systemic lupus erythematosus (JSLE) and associated them with disease duration and activity, use of medication (chloroquine and glucocorticoids), vitamin D intake, calcium and alkaline phosphatase levels, and bone mineral density. Thirty patients with JSLE were evaluated and compared to 30 healthy individuals, who were age and gender matched. Assessment was performed of clinical status, disease activity, anthropometry, laboratory markers, and bone mineral density. The 30 patients included 25 (83.3%) females and 16 (53.3%) Caucasians, with a mean age of 13.7 years. The mean age at diagnosis was 10.5 years and mean disease duration was 3.4 years. Mean levels of calcium, albumin, and alkaline phosphatase were significantly lower in patients with JSLE compared with controls (P<0.001, P=0.006, and P<0.001, respectively). Twenty-nine patients (97%) and 23 controls (77%) had 25(OH)D concentrations lower than 32 ng/mL, with significant differences between them (P<0.001). Fifteen patients (50%) had vitamin D levels <20 ng/mL and 14 had vitamin D levels between 20 and 32 ng/mL. However, these values were not associated with greater disease activity, higher levels of parathormone, medication intake, or bone mineral density. Vitamin D concentrations were similar with regard to ethnic group, body mass index, height for age, and pubertal stage. Significantly more frequently than in controls, we observed insufficient serum concentrations of 25(OH)D in patients with JSLE; however, we did not observe any association with disease activity, higher levels of parathormone, lower levels of alkaline phosphatase, use of medications, or bone mineral density alterations.


Assuntos
Conservadores da Densidade Óssea/uso terapêutico , Lúpus Eritematoso Sistêmico/sangue , Vitamina D/análogos & derivados , Vitamina D/uso terapêutico , Adolescente , Fosfatase Alcalina/sangue , Antirreumáticos/uso terapêutico , Densidade Óssea , Cálcio/sangue , Criança , Cloroquina/uso terapêutico , Estudos Transversais , Feminino , Glucocorticoides/uso terapêutico , Humanos , Medições Luminescentes , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Hormônio Paratireóideo/sangue , Albumina Sérica/análise , Estatísticas não Paramétricas , Vitamina D/sangue , População Branca , Adulto Jovem
5.
Braz J Med Biol Res ; 46(1): 98-102, 2013 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-23314341

RESUMO

Our objective was to evaluate the concentrations of serum 25-hydroxyvitamin D [25(OH)D], serum calcium, serum phosphorus, alkaline phosphatase, and parathormone (PTH) in patients with polyarticular juvenile idiopathic arthritis (JIA) and to associate them with disease duration and activity, bone mineral density and use of medications. In a cross-sectional and controlled study, 30 patients with polyarticular JIA were evaluated and compared to 30 healthy individuals matched for age and gender. Clinical status, anthropometry, laboratory markers in both patients and controls, and bone mineral density, only in the patients, were measured. Of the 30 patients included in the study, 23 (76.7%) were female and 16 (53.3%) non-Caucasian; mean age was 14 years (range = 4 to 20 years). Mean disease duration was 5 years (range = 1 to 12 years). The mean concentrations of serum albumin-corrected calcium (9.04 ± 0.41 mg/dL) and alkaline phosphatase (153.3 ± 100.1 IU) were significantly lower in patients with JIA than in controls (P < 0.0001 and P = 0.001, respectively). No differences in 25(OH)D, PTH or serum phosphorus were observed between JIA and control subjects. Regarding 25(OH)D concentration, 8 patients (26.7%) and 5 controls (16.7%) had 25(OH)D concentrations compatible with deficiency (lower than 20 ng/mL) and 14 patients (46.7%) and 18 controls (60%) had concentrations compatible with insufficiency (20-32 ng/mL). These values were not associated with disease activity, use of medications or bone mineral density. We observed a high frequency of 25(OH)D insufficiency and deficiency in the study sample. The compromised bone metabolism emphasizes the importance of follow-up of JIA patients.


Assuntos
Artrite Juvenil/sangue , Densidade Óssea , Osso e Ossos/metabolismo , Vitamina D/análogos & derivados , Adolescente , Fosfatase Alcalina/sangue , Artrite Juvenil/metabolismo , Biomarcadores/sangue , Cálcio/sangue , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos Transversais , Feminino , Humanos , Masculino , Hormônio Paratireóideo/sangue , Fosfatos/sangue , Vitamina D/sangue , Adulto Jovem
6.
Braz. j. med. biol. res ; 46(1): 98-102, 11/jan. 2013. tab
Artigo em Inglês | LILACS | ID: lil-665795

RESUMO

Our objective was to evaluate the concentrations of serum 25-hydroxyvitamin D [25(OH)D], serum calcium, serum phosphorus, alkaline phosphatase, and parathormone (PTH) in patients with polyarticular juvenile idiopathic arthritis (JIA) and to associate them with disease duration and activity, bone mineral density and use of medications. In a cross-sectional and controlled study, 30 patients with polyarticular JIA were evaluated and compared to 30 healthy individuals matched for age and gender. Clinical status, anthropometry, laboratory markers in both patients and controls, and bone mineral density, only in the patients, were measured. Of the 30 patients included in the study, 23 (76.7%) were female and 16 (53.3%) non-Caucasian; mean age was 14 years (range = 4 to 20 years). Mean disease duration was 5 years (range = 1 to 12 years). The mean concentrations of serum albumin-corrected calcium (9.04 ± 0.41 mg/dL) and alkaline phosphatase (153.3 ± 100.1 IU) were significantly lower in patients with JIA than in controls (P < 0.0001 and P = 0.001, respectively). No differences in 25(OH)D, PTH or serum phosphorus were observed between JIA and control subjects. Regarding 25(OH)D concentration, 8 patients (26.7%) and 5 controls (16.7%) had 25(OH)D concentrations compatible with deficiency (lower than 20 ng/mL) and 14 patients (46.7%) and 18 controls (60%) had concentrations compatible with insufficiency (20-32 ng/mL). These values were not associated with disease activity, use of medications or bone mineral density. We observed a high frequency of 25(OH)D insufficiency and deficiency in the study sample. The compromised bone metabolism emphasizes the importance of follow-up of JIA patients.


Assuntos
Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Adulto Jovem , Artrite Juvenil/sangue , Densidade Óssea , Osso e Ossos/metabolismo , Vitamina D/análogos & derivados , Fosfatase Alcalina/sangue , Artrite Juvenil/metabolismo , Biomarcadores/sangue , Estudos de Casos e Controles , Estudos Transversais , Cálcio/sangue , Hormônio Paratireóideo/sangue , Fosfatos/sangue , Vitamina D/sangue
7.
Br J Radiol ; 84(1003): 621-8, 2011 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-21081568

RESUMO

OBJECTIVE: This study aimed to estimate the prevalence of osteonecrosis (ON) in juvenile systemic lupus erythematosus (SLE) patients using joint-specific and whole-body MRI; to explore risk factors that are associated with the development of ON; and to evaluate prospectively patients 1 year after initial imaging. METHOD: Within a 2 year period, we studied 40 juvenile SLE patients (aged 8-18 years) with a history of steroid use of more than 3 months duration. Risk factors including disease activity, corticosteroid use, vasculitis, Raynaud's phenomenon and lipid profile were evaluated. All patients underwent MRI of the hips, knees and ankles using joint-specific MRI. Whole-body STIR (short tau inversion recovery) MRI was performed in all patients with ON lesions. RESULTS: Osteonecrosis was identified in 7 patients (17.5 %) upon joint-specific MRI. Whole-body STIR MRI detected ON in 6 of these 7 patients. There was no significant difference between the ON and non-ON groups in the risk factors studied. One patient had pre-existing symptomatic ON. At 1 year follow-up, the ON lesions had resolved in one patient, remained stable in four and decreased in size in two. No asymptomatic patients with ON developed clinical manifestations. CONCLUSION: Whole-body STIR MRI may be useful in detecting ON lesions in juvenile SLE patients but larger studies are needed to define its role.


Assuntos
Articulação do Tornozelo , Articulação do Joelho , Lúpus Eritematoso Sistêmico/complicações , Imageamento por Ressonância Magnética/métodos , Osteonecrose/diagnóstico , Imagem Corporal Total/métodos , Adolescente , Criança , Feminino , Humanos , Masculino , Prevalência , Estudos Prospectivos , Fatores de Risco
9.
Clin Exp Rheumatol ; 27(6): 1031-8, 2009.
Artigo em Inglês | MEDLINE | ID: mdl-20149327

RESUMO

OBJECTIVE: To describe onset features, classification and treatment of juvenile dermatomyositis (JDM) and juvenile polymyositis (JPM) from a multicentre registry. METHODS: Inclusion criteria were onset age lower than 18 years and a diagnosis of any idiopathic inflammatory myopathy (IIM) by attending physician. Bohan & Peter (1975) criteria categorisation was established by a scoring algorithm to define JDM and JPM based on clinical protocol data. RESULTS: Of the 189 cases included, 178 were classified as JDM, 9 as JPM (19.8: 1) and 2 did not fit the criteria; 6.9% had features of chronic arthritis and connective tissue disease overlap. Diagnosis classification agreement occurred in 66.1%. Median onset age was 7 years, median follow-up duration was 3.6 years. Malignancy was described in 2 (1.1%) cases. Muscle weakness occurred in 95.8%; heliotrope rash 83.5%; Gottron plaques 83.1%; 92% had at least one abnormal muscle enzyme result. Muscle biopsy performed in 74.6% was abnormal in 91.5% and electromyogram performed in 39.2% resulted abnormal in 93.2%. Logistic regression analysis was done in 66 cases with all parameters assessed and only aldolase resulted significant, as independent variable for definite JDM (OR=5.4, 95%CI 1.2-24.4, p=0.03). Regarding treatment, 97.9% received steroids; 72% had in addition at least one: methotrexate (75.7%), hydroxychloroquine (64.7%), cyclosporine A (20.6%), IV immunoglobulin (20.6%), azathioprine (10.3%) or cyclophosphamide (9.6%). In this series 24.3% developed calcinosis and mortality rate was 4.2%. CONCLUSION: Evaluation of predefined criteria set for a valid diagnosis indicated aldolase as the most important parameter associated with definite JDM category. In practice, prednisone-methotrexate combination was the most indicated treatment.


Assuntos
Dermatomiosite/classificação , Dermatomiosite/diagnóstico , Adolescente , Idade de Início , Brasil , Criança , Pré-Escolar , Dermatomiosite/tratamento farmacológico , Progressão da Doença , Feminino , Glucocorticoides/uso terapêutico , Humanos , Lactente , Masculino , Seleção de Pacientes , Sistema de Registros , Análise de Regressão , Índice de Gravidade de Doença , Resultado do Tratamento
10.
Lupus ; 16(7): 531-6, 2007.
Artigo em Inglês | MEDLINE | ID: mdl-17670855

RESUMO

We evaluated the prevalence and clinical associations of amenorrhea in 298 female juvenile systemic lupus erythematosus (JSLE) patients (ACR criteria) followed in 12 Brazilian Paediatric Rheumatology centres. Amenorrhea was observed in 35 patients (11.7%) with a mean duration of 7.2 +/- 3.6 months. The hormones were performed in 32/35 patients and none of them had FSH and LH levels above and estradiol below the normal range according to pubertal changes. JSLE patients with amenorrhea were younger (15.04 +/- 2.5 versus 17.8 +/- 3.1 years; P = 0.001), and had a shorter period of time between menarche and current age (3.4 +/- 2.9 versus 6.7 +/- 5.4 years; P = 0.001). Interestingly, the frequency, cumulative dose, number of pulses and duration of intravenous cyclophosphamide treatment were alike in patients with and without amenorrhea (P > 0.05). In contrast, patients with amenorrhea had significantly higher SLEDAI (P = 0.01) and SLICC/ACR-DI (P = 0.024) scores compared to those without this condition. Independent risk factors identified by multivariate analysis were higher SLEDAI (OR = 1.059; CI = 1.004-1.116; P = 0.034) and SLICC/ACR-DI (OR = 2.125; IC = 1.373-3.291; P = 0.001) scores. Our data suggest that in spite of immunosuppressive therapy, JSLE patients have an adequate ovarian follicular reserve and amenorrhea is particularly associated with disease activity and damage.


Assuntos
Amenorreia/etiologia , Lúpus Eritematoso Sistêmico/complicações , Adolescente , Adulto , Idade de Início , Amenorreia/sangue , Amenorreia/epidemiologia , Biomarcadores/sangue , Brasil/epidemiologia , Criança , Estradiol/sangue , Feminino , Fluorimunoensaio , Hormônio Foliculoestimulante/sangue , Seguimentos , Humanos , Incidência , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/epidemiologia , Hormônio Luteinizante/sangue , Ciclo Menstrual/sangue , Prevalência , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença
11.
Clin Exp Rheumatol ; 24(5): 594-8, 2006.
Artigo em Inglês | MEDLINE | ID: mdl-17181933

RESUMO

OBJECTIVE: Studies in adults with SLE have evidenced increase of homocysteine related, mainly, to thromboembolic events. The aim of our study was to evaluate plasma homocysteine concentration in children with systemic lupus erythematosus (SLE) and its correlation with renal involvement, serum and erythrocyte folate, vitamin B12, antiphospholipid antibodies, estimated creatinine clearance and dyslipidemia. METHODS: Thirty-two children (29 females) with SLE and 32 healthy controls (29 females) matched for age and sex were included in the study. The mean age of patients and controls was 14.2 years (range from 10 to 18 years). Only one patient presented one thrombotic event. Plasma homocysteine, erythrocyte and serum folate, vitamin B12, lipid profile, antiphospholipid antibodies and estimated creatinine clearance were evaluated. Raised homocysteine concentration was defined as equal or more than 12.9 mol/L. RESULTS: Raised homocysteine concentration was detected in 15 (46.9%) children with SLE with an important statistical difference in relation to control group (p < 0.001). A positive correlation was found between plasma homocysteine concentration and renal involvement (odds ratio 11.1 [95% CI 1.50-82.24], p = 0.01) based on the presence of renal biopsy, abnormalities of urine sediment and/or serum creatinine. However, when we performed the estimated creatinine clearance the correlation with homocysteine concentration was not positive. We did not observe abnormalities in serum and erythrocyte folate and vitamin B12 in our patients. However, they presented significant higher concentrations of TC total cholesterol (p = 0.005) and of LDL low-density lipoprotein (p = 0.02) than controls. CONCLUSION: Elevated plasma homocysteine concentration is frequent in children with SLE. We believe that these results may signalize to the possibility of complications in our patients later in life. Further long-term and prospective studies are needed in order to determine the real role of the homocysteine concentration as a risk factor in children.


Assuntos
Homocisteína/sangue , Lúpus Eritematoso Sistêmico/sangue , Adolescente , Criança , Colesterol/sangue , Estudos Transversais , Eritrócitos/química , Feminino , Ácido Fólico/sangue , Humanos , Testes de Função Renal , Lipoproteínas LDL/sangue , Lúpus Eritematoso Sistêmico/complicações , Nefrite Lúpica/sangue , Nefrite Lúpica/fisiopatologia , Masculino
12.
Clin Exp Rheumatol ; 24(5): 599-605, 2006.
Artigo em Inglês | MEDLINE | ID: mdl-17181934

RESUMO

OBJECTIVE: To investigate the clinical use patterns, clinical effect and safety of cyclosporine A (CSA) in juvenile idiopathic arthritis (JIA) in the setting of routine clinical care. METHODS: An open-ended, phase IV post marketing surveillance study was conducted among members of the Pediatric Rheumatology Collaborative Study Group (PRCSG) and of the Paediatric Rheumatology International Trials Organisation (PRINTO) to identify patients with polyarticular course JIA who had received CSA during the course of their disease. RESULTS: A total of 329 patients, half of whom had systemic JIA, were collected in 21 countries. Data were collected during 1240 routine clinic visits. CSA was started at a mean of 5.8 years after disease onset and was given at a mean dose of 3.4 mg/kg/day. The drug was administered in combination with MTX in 61% and along with prednisone in 65% of the patients who were still receiving CSA. Among patients who were still receiving CSA therapy at the last reported visit, remission was documented in 9% of the patients, whereas in 61% of the patients the disease activity was rated as moderate or severe. The most frequent reason for discontinuation of CSA was insufficient therapeutic effect (61% of the patients); only 10% of the patients stopped CSA because of remission. In 17% of the patients, side effects of therapy was given as the primary reason for discontinuation. CONCLUSION: This survey suggests that CSA may have a less favourable efficacy profile than MTX and etanercept, whereas the frequency of side effects may be similar. The exact place of CSA in the treatment of JIA can only be established via controlled clinical trial.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Ciclosporina/uso terapêutico , Vigilância de Produtos Comercializados , Artrite Juvenil/fisiopatologia , Criança , Quimioterapia Combinada , Nível de Saúde , Humanos , Metotrexato/uso terapêutico , Prednisona/uso terapêutico , Indução de Remissão , Índice de Gravidade de Doença
13.
Rheumatology (Oxford) ; 45(5): 614-20, 2006 May.
Artigo em Inglês | MEDLINE | ID: mdl-16368732

RESUMO

OBJECTIVE: Juvenile localized scleroderma (JLS) includes a number of conditions often grouped together. With the long-term goal of developing uniform classification criteria, we studied the epidemiological, clinical and immunological features of children with JLS followed by paediatric rheumatology and dermatology centres. METHODS: A large, multicentre, multinational study was conducted by collecting information on the demographics, family history, triggering environmental factors, clinical and laboratory features, and treatment of patients with JLS. RESULTS: Seven hundred and fifty patients with JLS from 70 centres were enrolled into the study. The disease duration at diagnosis was 18 months. Linear scleroderma (LS) was the most frequent subtype (65%), followed by plaque morphea (PM) (26%), generalized morphea (GM) (7%) and deep morphea (DM) (2%). As many as 15% of patients had a mixed subtype. Ninety-one patients (12%) had a positive family history for rheumatic or autoimmune diseases; 100 (13.3%) reported environmental events as possible trigger. ANA was positive in 42.3% of the patients, with a higher prevalence in the LS-DM subtype than in the PM-GM subtype. Scl70 was detected in the sera of 3% of the patients, anticentromere antibody in 2%, anti-double-stranded DNA in 4%, anti-cardiolipin antibody in 13% and rheumatoid factor in 16%. Methotrexate was the drug most frequently used, especially during the last 5 yr. CONCLUSION: This study represents the largest collection of patients with JLS ever reported. The insidious onset of the disease, the delay in diagnosis, the recognition of mixed subtype and the better definition of the other subtypes should influence our efforts in educating trainees and practitioners and help in developing a comprehensive classification system for this syndrome.


Assuntos
Esclerodermia Localizada/diagnóstico , Adolescente , Idade de Início , Autoanticorpos/sangue , Doenças Autoimunes/genética , Criança , Pré-Escolar , Meio Ambiente , Feminino , Predisposição Genética para Doença , Humanos , Imunossupressores/uso terapêutico , Lactente , Recém-Nascido , Cooperação Internacional , Masculino , Metotrexato/uso terapêutico , Doenças Reumáticas/genética , Fatores de Risco , Esclerodermia Localizada/tratamento farmacológico , Esclerodermia Localizada/epidemiologia , Esclerodermia Localizada/etiologia
14.
Braz J Med Biol Res ; 38(2): 309-13, 2005 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-15785843

RESUMO

We measured bone mineral density (BMD) in girls with juvenile dermatomyositis (JDM) considering multiple factors in order to determine if it could be used as a predictor of reduction in bone mass. A cross-sectional study of lumbar spine BMD (L2-L4) was conducted on 10 girls aged 7-16 years with JDM. A group of 20 age-matched healthy girls was used as control. Lumbar spine BMD was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in all patients and controls. Duration of disease and mean daily and cumulative steroid doses were calculated for all patients on the basis of their medical charts. JDM activity was determined on the basis of the presence of muscle weakness, cutaneous vasculitis and/or elevation of serum concentration of one or more skeletal muscle enzymes. Seven patients demonstrated osteopenia or osteoporosis. Lumbar BMD was significantly lower in the JDM patients than the age-matched healthy control girls (0.712 vs 0.878, respectively; Student t-test, P = 0.041). No significant correlation between BMD and age, height, Tanner stage, disease duration, corticosteroid use, or disease activity was observed in JDM girls, but a correlation was observed between BMD and weight (Pearson's correlation coefficient, r = 0.802). Patients with JDM may be at risk for a significant reduction in BMD that might contribute to further skeletal fragility. Our results suggest that reduced bone mass in JDM may be related to other intrinsic mechanisms in addition to steroid treatment and some aspects of the disease itself may contribute to this condition.


Assuntos
Densidade Óssea , Doenças Ósseas Metabólicas/complicações , Dermatomiosite/complicações , Absorciometria de Fóton , Adolescente , Doenças Ósseas Metabólicas/diagnóstico por imagem , Estudos de Casos e Controles , Criança , Estudos Transversais , Dermatomiosite/diagnóstico por imagem , Feminino , Humanos , Vértebras Lombares/diagnóstico por imagem , Osteoporose/complicações , Osteoporose/diagnóstico por imagem
15.
Braz. j. med. biol. res ; 38(2): 309-313, fev. 2005. tab
Artigo em Inglês | LILACS | ID: lil-393648

RESUMO

We measured bone mineral density (BMD) in girls with juvenile dermatomyositis (JDM) considering multiple factors in order to determine if it could be used as a predictor of reduction in bone mass. A cross-sectional study of lumbar spine BMD (L2-L4) was conducted on 10 girls aged 7-16 years with JDM. A group of 20 age-matched healthy girls was used as control. Lumbar spine BMD was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in all patients and controls. Duration of disease and mean daily and cumulative steroid doses were calculated for all patients on the basis of their medical charts. JDM activity was determined on the basis of the presence of muscle weakness, cutaneous vasculitis and/or elevation of serum concentration of one or more skeletal muscle enzymes. Seven patients demonstrated osteopenia or osteoporosis. Lumbar BMD was significantly lower in the JDM patients than the age-matched healthy control girls (0.712 vs 0.878, respectively; Student t-test, P = 0.041). No significant correlation between BMD and age, height, Tanner stage, disease duration, corticosteroid use, or disease activity was observed in JDM girls, but a correlation was observed between BMD and weight (Pearson's correlation coefficient, r = 0.802). Patients with JDM may be at risk for a significant reduction in BMD that might contribute to further skeletal fragility. Our results suggest that reduced bone mass in JDM may be related to other intrinsic mechanisms in addition to steroid treatment and some aspects of the disease itself may contribute to this condition.


Assuntos
Humanos , Feminino , Criança , Adolescente , Densidade Óssea , Doenças Ósseas Metabólicas/complicações , Dermatomiosite/complicações , Absorciometria de Fóton , Doenças Ósseas Metabólicas , Estudos de Casos e Controles , Estudos Transversais , Dermatomiosite , Vértebras Lombares , Osteoporose/complicações , Osteoporose
18.
Artigo em Inglês | MEDLINE | ID: mdl-12968394

RESUMO

OBJECTIVES: (1) To study the correlation among conventional clinical and laboratory parameters and the relation between the number of lymphocytes and neutrophils (L/N) in cell suspensions from peripheral blood of patients with juvenile idiopathic arthritis (JIA). (2) To evaluate the L/N relation of JIA patients after an 8 year follow-up period. METHODS: Fifty-one JIA patients (25 female, disease course: 19 systemic, 15 polyarticular, 17 pauciarticular) were enrolled in the study. To measure the L/N relation, we used Boyum's method: The leucocyte separation was done by centrifugation of peripheral blood on Ficoll-Hypaque (FH) gradient, and the number of lymphocytes, monocytes, and neutrophils in 500 cells was determined. The following clinical and laboratory parameters were evaluated: disease activity, number of active and limited joints, functional capacity, erythrocyte sedimentation rate (ESR), and C reactive protein (CRP). Twenty-four healthy children were used as controls. We also studied 13/51 patients from our Pediatric Rheumatology Unit who had been evaluated by the same method 8 years before. RESULTS: We observed the lowest L/N relation in patients with active disease, especially those with polyarticular course. A statistical correlation was also observed with the acute-phase reactants (ESR and CRP, p < 0.05). The majority of patients who had presented a low L/N relation at the first evaluation (8 years before) had a worse outcome. CONCLUSION: The measure of L/N relation from peripheral blood could be used as an auxiliary tool in the assessment of the activity and outcome of JIA patients, especially at disease onset.


Assuntos
Artrite Juvenil/sangue , Neutrófilos/citologia , Adolescente , Artrite Juvenil/diagnóstico , Sedimentação Sanguínea , Proteína C-Reativa/análise , Separação Celular , Criança , Feminino , Seguimentos , Humanos , Linfócitos/citologia , Masculino , Monócitos/citologia
19.
Eur J Clin Nutr ; 57(5): 706-12, 2003 May.
Artigo em Inglês | MEDLINE | ID: mdl-12771972

RESUMO

OBJECTIVE: To evaluate the copper and zinc intake and serum levels in patients with juvenile rheumatoid arthritis (JRA), considering the pauci and polyarticular types, the disease activity and duration, the number of inflamed joints and the use of corticosteroids therapy. DESIGN: Cross-sectional study with control group. SETTING: Outpatients of the pediatric rheumatology public health clinic, of the Universidade Federal de São Paulo/Escola Paulista de Medicina, Brazil. SUBJECTS: Forty-one patients with JRA were evaluated and 23 patients' brothers, as a control group. INTERVENTIONS: Copper and zinc intake evaluation by Food Register method. Copper and zinc serum levels by atomic absorption spectrophotometry. RESULTS: The disease activity did not determine difference in copper (P=0.624) and zinc (P=0.705) intake, being predominantly below the Recommended Dietary Allowances. The serum copper in relation to control was statistically greater (P=0.018), showing that the number of inflamed joints is statistically significantly related with its variation (P=0.001). The serum zinc was not different either in relation to control (P=0.940) or to the disease characteristics. CONCLUSIONS: The evaluation of copper intake seems to be of fundamental importance. It may influence the efficiency of the organic serum response. More research is needed to indicate, with security, adequate zinc intake.


Assuntos
Artrite Juvenil/sangue , Cobre/administração & dosagem , Cobre/sangue , Dieta , Zinco/administração & dosagem , Zinco/sangue , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Modelos Lineares , Masculino , Valores de Referência , Caracteres Sexuais
20.
Braz J Med Biol Res ; 35(10): 1159-63, 2002 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-12424488

RESUMO

We evaluated spine bone mineral density (BMD) in Brazilian children with juvenile systemic lupus erythematosus (JSLE) in order to detect potential predictors of reduction in bone mass. A cross-sectional study of BMD at the lumbar spine level (L2-L4) was conducted on 16 female JSLE patients aged 6-17 years. Thirty-two age-matched healthy girls were used as control. BMD at the lumbar spine was measured by dual-energy X-ray absorptiometry. Weight, height and pubertal Tanner stage were determined in patients and controls. Disease duration, mean daily steroid doses, mean cumulative steroid doses and JSLE activity measured by the systemic lupus erythematosus disease activity index (SLEDAI) were determined for all JSLE patients based on their medical charts. All parameters were used as potential determinant factors for bone loss. Lumbar BMD tended to be lower in the JSLE patients, however, this difference was not statistically significant (P = 0.10). No significant correlation was observed in JSLE girls between BMD and age, height, Tanner stage, disease duration, corticosteroid use or disease activity. We found a weak correlation between BMD and weight (r = 0.672). In the JSLE group we found no significant parameters to correlate with reduced bone mass. Disease activity and mean cumulative steroid doses were not related to BMD values. We did not observe reduced bone mass in female JSLE.


Assuntos
Densidade Óssea , Lúpus Eritematoso Sistêmico/fisiopatologia , Absorciometria de Fóton , Adolescente , Corticosteroides/efeitos adversos , Peso Corporal , Estudos de Casos e Controles , Criança , Estudos Transversais , Feminino , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Fatores de Risco
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