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 EvidênciasRESUMO
SETTING: As conclusive data on the performance of interferon-gamma release assays (IGRAs) in paediatric TB are lacking, many guidelines do not recommend their use for TB diagnosis in this population in Brazil. OBJECTIVE: To evaluate the performance of an IGRA by investigating its concordance with the tuberculin skin test (TST) and the role of IGRAs in clinical management and treatment outcomes in children with TB. DESIGN: A historic cohort study was used to evaluate the performance of T-SPOT®.TB (ELISpot) and other tests, such as the TST, in paediatric patients with or without immunodeficiency who were under investigation for latent tuberculous infection (LTBI) or active tuberculosis (TB). RESULTS: Of 86 paediatric patients evaluated, 41 (48%) were immunocompetent and 45 (52%) immunocompromised. All patients underwent T-SPOT.TB, while 63 underwent both ELISpot and TST; test results were concordant in 50 patients (79.4%): 22/31 (71%) in immunocompetent (κ = 0.418, P = 0.02) and 28/32 (87.5%) in immunocompromised patients (κ = 0.526, P = 0.003). TB was diagnosed on the basis of the ELISpot result in 21% (18/86) cases; the contribution of the ELISpot assay was greater in immunocompetent patients than in those who were immunocompromised (13/41, 31.7% vs. 5/45, 11.1%, χ2 P = 0.038). CONCLUSION: ELISpot and TST results were moderately concordant in both groups of patients. ELISpot contribution was higher among immunocompetent patients than among immunocompromised patients.
Assuntos
ELISPOT/estatística & dados numéricos , Testes de Liberação de Interferon-gama/estatística & dados numéricos , Teste Tuberculínico/estatística & dados numéricos , Tuberculose/diagnóstico , Adolescente , Brasil , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Hospedeiro Imunocomprometido , Lactente , Masculino , Adulto JovemRESUMO
Objective The objective of this study was to compare demographic data, clinical/laboratorial features and disease activity at diagnosis in three different groups with distinct time intervals between onset of signs/symptoms and disease diagnosis. Methods A multicenter study was performed in 1555 childhood-onset systemic lupus erythematosus (American College of Rheumatology criteria) patients from 27 pediatric rheumatology services. Patients were divided into three childhood-onset systemic lupus erythematosus groups: A: short time interval to diagnosis (<1 month); B: intermediate time interval (≥1 and <3 months); and C: long time interval (≥3 months). An investigator meeting was held to define the protocol. Demographic data, SLICC classification criteria and SLEDAI-2 K were evaluated. Results The number of patients in each group was: A = 60 (4%); B = 522 (33.5%); and C = 973 (62.5%). The median age at diagnosis (11.1 (4.2-17) vs. 12 (1.9-17.7) vs. 12.5 (3-18) years, P = 0.025) was significantly lower in group A compared with groups B and C. The median number of diagnostic criteria according to SLICC (7 (4-12) vs. 6 (4-13) vs. 6 (4-12), P < 0.0001) and SLEDAI-2 K (18 (6-57) vs. 16 (2-63) vs. 13 (1-49), P < 0.0001) were significantly higher in group A than the other two groups. The frequency of oral ulcers in the palate (25% vs. 15% vs. 11%, P = 0.003), pleuritis (25% vs. 24% vs. 14%, P < 0.0001), nephritis (52% vs. 47% vs. 40%, P = 0.009), neuropsychiatric manifestations (22% vs. 13% vs. 10%, P = 0.008), thrombocytopenia (32% vs. 18% vs. 19%, P = 0.037), leucopenia/lymphopenia (65% vs. 46% vs. 40%, P < 0.0001) and anti-dsDNA antibodies (79% vs. 66% vs. 61%, P = 0.01) were significantly higher in group A compared with the other groups. In contrast, group C had a less severe disease characterized by higher frequencies of synovitis (61% vs. 66% vs. 71%, P = 0.032) and lower frequencies of serositis (37% vs. 33% vs. 25%, P = 0.002), proteinuria >500 mg/day (48% vs. 45% vs. 36%, P = 0.002) and low complement levels (81% vs. 81% vs. 71%, P < 0.0001) compared with groups A or B. Conclusions Our large Brazilian multicenter study demonstrated that for most childhood-onset systemic lupus erythematosus patients, diagnosis is delayed probably due to mild disease onset. Conversely, the minority has a very short time interval to diagnosis and a presentation with a more severe and active multisystemic condition.
Assuntos
Diagnóstico Tardio , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/epidemiologia , Adolescente , Idade de Início , Biomarcadores/sangue , Brasil/epidemiologia , Criança , Pré-Escolar , Progressão da Doença , Feminino , Humanos , Lúpus Eritematoso Sistêmico/sangue , Masculino , Valor Preditivo dos Testes , Prognóstico , Estudos Retrospectivos , Fatores de Risco , Índice de Gravidade de Doença , Fatores de TempoRESUMO
Objective This study sought to evaluate the effects of a nutritional intervention on the lipid metabolism biomarkers associated with cardiovascular risk, and their variation over time, in juvenile systemic lupus erythematosus (JSLE) patients. This study also investigated the relationships between these biomarkers and dietary intake, nutritional status, disease variables, and medication used. Methods A total of 31 10- to 19-year-old female adolescents with JSLE for at least six months were analyzed. The participants were randomly allocated to two groups: nutritional intervention or control. The intervention group received verbal and printed nutritional instructions once per month over nine months. Before and after the intervention, the participants underwent assessments of anthropometry; dietary intake; physical activity; socioeconomic status; total cholesterol and fractions; triglycerides; apolipoprotein A (Apo A-I); apolipoprotein B (Apo B); paraoxonase (PON) activity (a) and amount (q); myeloperoxidase (MPO); and small, dense LDL-c (sdLDL) particles. Results After nine months, we found significant reductions in the calorie, carbohydrate, total fat, saturated fat, and trans fat intakes in the intervention compared with the control group over time. The PONa/HDL-c ratio increased by 3.18 U/ml/mg/dl in the intervention group and by 0.63 U/ml/mg/dl in the control group ( p = 0.037). Unlike the intervention group, the sdLDL levels of the control group worsened over time ( p = 0.018). Conclusion The present study detected a reduction in calorie and fat intake, which indicates an improvement of HDL-c function and possible protection against cardiovascular risk for the intervention group.
Assuntos
Dieta Saudável , Dislipidemias/dietoterapia , Lipídeos/sangue , Lúpus Eritematoso Sistêmico/dietoterapia , Estado Nutricional , Folhetos , Educação de Pacientes como Assunto/métodos , Adolescente , Fatores Etários , Biomarcadores/sangue , Brasil , Doenças Cardiovasculares/prevenção & controle , Criança , Dislipidemias/sangue , Dislipidemias/diagnóstico , Dislipidemias/fisiopatologia , Ingestão de Energia , Comportamento Alimentar , Feminino , Conhecimentos, Atitudes e Prática em Saúde , Humanos , Lúpus Eritematoso Sistêmico/sangue , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/fisiopatologia , Fatores de Risco , Fatores Sexuais , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody. Methods This was a retrospective multicenter study performed in 10 pediatric rheumatology services of São Paulo state, Brazil. Anti-P antibodies were measured by ELISA in 228 cSLE patients. Results Anti-P antibodies were observed in 61/228 (27%) cSLE patients. Frequencies of cumulative lymphadenopathy (29% vs. 15%, p = 0.014), acute confusional state (13% vs. 5%, p = 0.041), mood disorder (18% vs. 8%, p = 0.041), autoimmune hemolytic anemia (34% vs. 15%, p = 0.001), as well as presence of anti-Sm (67% vs. 40%, p = 0.001), anti-RNP (39% vs. 21%, p = 0.012) and anti-Ro/SSA antibodies (43% vs. 25%, p = 0.016) were significantly higher in cSLE patients with anti-P antibodies compared to those without these autoantibodies. A multiple regression model revealed that anti-P antibodies were associated with autoimmune hemolytic anemia (odds ratio (OR) = 2.758, 95% confidence interval (CI): 1.304-5.833, p = 0.008) and anti-Sm antibody (OR = 2.719, 95% CI: 1.365-5.418, p = 0.004). The SLICC/ACR damage index was comparable in patients with and without anti-P antibodies ( p = 0.780). Conclusions The novel association of anti-P antibodies and autoimmune hemolytic anemia was evidenced in cSLE patients and further studies are necessary to determine if anti-P titers may vary with this hematological manifestation.
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Anemia Hemolítica Autoimune/epidemiologia , Autoanticorpos/metabolismo , Lúpus Eritematoso Sistêmico/complicações , Transtornos do Humor/epidemiologia , Proteínas Ribossômicas/imunologia , Adolescente , Idade de Início , Anemia Hemolítica Autoimune/imunologia , Criança , Pré-Escolar , Feminino , Humanos , Lúpus Eritematoso Sistêmico/imunologia , Lúpus Eritematoso Sistêmico/psicologia , Masculino , Análise de Regressão , Estudos Retrospectivos , Adulto JovemRESUMO
Objective The objective of this study was to assess outcomes of childhood systemic lupus erythematosus (cSLE) in three different age groups evaluated at last visit: group A early-onset disease (<6 years), group B school age (≥6 and <12 years) and group C adolescent (≥12 and <18 years). Methods An observational cohort study was performed in ten pediatric rheumatology centers, including 847 cSLE patients. Results Group A had 39 (4%), B 395 (47%) and C 413 (49%). Median disease duration was significantly higher in group A compared to groups B and C (8.3 (0.1-23.4) vs 6.2 (0-17) vs 3.3 (0-14.6) years, p < 0.0001). The median Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) (0 (0-9) vs 0 (0-6) vs 0 (0-7), p = 0.065) was comparable in the three groups. Further analysis of organ/system damage revealed that frequencies of neuropsychiatric (21% vs 10% vs 7%, p = 0.007), skin (10% vs 1% vs 3%, p = 0.002) and peripheral vascular involvements (5% vs 3% vs 0.3%, p = 0.008) were more often observed in group A compared to groups B and C. Frequencies of severe cumulative lupus manifestations such as nephritis, thrombocytopenia, and autoimmune hemolytic anemia were similar in all groups ( p > 0.05). Mortality rate was significantly higher in group A compared to groups B and C (15% vs 10% vs 6%, p = 0.028). Out of 69 deaths, 33/69 (48%) occurred within the first two years after diagnosis. Infections accounted for 54/69 (78%) of the deaths and 38/54 (70%) had concomitant disease activity. Conclusions This large multicenter study provided evidence that early-onset cSLE group had distinct outcomes. This group was characterized by higher mortality rate and neuropsychiatric/vascular/skin organ damage in spite of comparable frequencies of severe cumulative lupus manifestations. We also identified that overall death in cSLE patients was an early event mainly attributed to infection associated with disease activity.
Assuntos
Anemia Hemolítica Autoimune/complicações , Lúpus Eritematoso Sistêmico/complicações , Nefrite/complicações , Trombocitopenia/complicações , Adolescente , Idade de Início , Anemia Hemolítica Autoimune/diagnóstico , Anemia Hemolítica Autoimune/patologia , Brasil/epidemiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Imunossupressores/uso terapêutico , Lactente , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/epidemiologia , Lúpus Eritematoso Sistêmico/mortalidade , Mortalidade , Nefrite/diagnóstico , Nefrite/epidemiologia , Nefrite/mortalidade , Gravidez , Estudos Retrospectivos , Índice de Gravidade de Doença , Trombocitopenia/diagnóstico , Trombocitopenia/patologia , Resultado do TratamentoRESUMO
OBJECTIVE: The aim of this multicenter study in a large childhood-onset systemic lupus erythematosus (cSLE) population was to assess the herpes zoster infection (HZI) prevalence, demographic data, clinical manifestations, laboratory findings, treatment, and outcome. METHODS: A retrospective multicenter cohort study (Brazilian cSLE group) was performed in ten Pediatric Rheumatology services in São Paulo State, Brazil, and included 852 cSLE patients. HZI was defined according to the presence of acute vesicular-bullous lesions on erythematous/edematous base, in a dermatomal distribution. Post-herpetic neuralgia was defined as persistent pain after one month of resolution of lesions in the same dermatome. Patients were divided in two groups for the assessment of current lupus manifestations, laboratory findings, and treatment: patients with HZI (evaluated at the first HZI) and patients without HZI (evaluated at the last visit). RESULTS: The frequency of HZI in cSLE patients was 120/852 (14%). Hospitalization occurred in 73 (61%) and overlap bacterial infection in 16 (13%). Intravenous or oral aciclovir was administered in 113/120 (94%) cSLE patients at HZI diagnosis. None of them had ophthalmic complication or death. Post-herpetic neuralgia occurred in 6/120 (5%). After Holm-Bonferroni correction for multiple comparisons, disease duration (1.58 vs 4.41 years, p < 0.0001) was significantly lower in HZI cSLE patients compared to those without HZI. Nephritis (37% vs 18%, p < 0.0001), lymphopenia (32% vs 17%, p < 0.0001) prednisone (97% vs 77%, p < 0.0001), cyclophosphamide (20% vs 5%, p < 0.0001) and SLE Disease Activity Index 2000 (6.0 (0-35) vs 2 (0-45), p < 0.0001) were significantly higher in the former group. The logistic regression model showed that four independent variables were associated with HZI: disease duration < 1 year (OR 2.893 (CI 1.821-4.597), p < 0.0001), lymphopenia <1500/mm(3) (OR 1.931 (CI 1.183-3.153), p = 0.009), prednisone (OR 6.723 (CI 2.072-21.815), p = 0.002), and cyclophosphamide use (OR 4.060 (CI 2.174-7.583), p < 0.0001). CONCLUSION: HZI is an early viral infection in cSLE with a typical dermatomal distribution. Lymphopenia and immunosuppressive treatment seem to be major factors underlying this complication in spite of a benign course.
Assuntos
Ciclofosfamida/efeitos adversos , Herpes Zoster/epidemiologia , Imunossupressores/efeitos adversos , Lúpus Eritematoso Sistêmico/complicações , Prednisona/efeitos adversos , Aciclovir/administração & dosagem , Adolescente , Adulto , Idade de Início , Antivirais/administração & dosagem , Brasil/epidemiologia , Criança , Pré-Escolar , Feminino , Herpes Zoster/tratamento farmacológico , Hospitalização/estatística & dados numéricos , Humanos , Lactente , Modelos Logísticos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Linfopenia/epidemiologia , Masculino , Nefrite/epidemiologia , Estudos Retrospectivos , Índice de Gravidade de Doença , Adulto JovemRESUMO
The aim of this study is to evaluate body composition and the bone mineral density in female adolescents with juvenile systemic lupus erythematosus. Body composition (BC) and bone mineral density (BMD) were evaluated in an observational cohort study with 35 postmenarcheal adolescent females. The variables studied were as follows: current and cumulative corticosteroid dose, intake of supplements containing calcium and vitamin D, 24-h proteinuria, body mass index (BMI), and height for age (Z-score). BC was assessed using dual-energy X-ray absorptiometry (DXA) at two time points (median interval of 1.2 years). The fat mass index (FMI = fat mass in kilograms divided by the height in meters squared) and lean mass index (LMI = lean mass in kilograms divided by the height in meters squared) were calculated based on the DXA results. BMD was classified according to the International Society of Clinical Densitometry (low BMD for chronological age < -2.0 standard deviations). .The mean age of the subjects was 15.4 ± 1.8 years. Of patients, 54.3 % were normal weight, 22.8 % were overweight, 22.8 % were obese, and 8.6 % had short stature. Low BMD for chronological age was observed in 42.8 % of patients, and 60 % were not taking vitamin D. There was no significant difference between the two time points with respect to FMI, LMI, or body mass index Z-score (ZBMI); however, BMD has decreased significantly (p = 0.011). There was an association between not taking a vitamin D supplement and decreased BMD (p = 0.027). Almost half of the patients had altered nutritional status. The BMD decrease in adolescents with juvenile systemic lupus erythematosus (JSLE) was associated with the lack of vitamin D supplementation, highlighting the importance of well-defined vitamin D supplementation protocols.
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Corticosteroides/uso terapêutico , Conservadores da Densidade Óssea/uso terapêutico , Suplementos Nutricionais/estatística & dados numéricos , Lúpus Eritematoso Sistêmico/epidemiologia , Osteoporose/epidemiologia , Vitamina D/uso terapêutico , Adolescente , Composição Corporal , Densidade Óssea , Doenças Ósseas Metabólicas/epidemiologia , Cálcio da Dieta/uso terapêutico , Estudos de Coortes , Feminino , Transtornos do Crescimento/epidemiologia , Humanos , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Masculino , Obesidade/epidemiologia , Sobrepeso/epidemiologiaRESUMO
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 DoençaRESUMO
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.