A5022573190- Autoimmune and Inflammatory Disorders Research
- Systemic Lupus Erythematosus Research
- Systemic Sclerosis and Related Diseases
- Adolescent and Pediatric Healthcare
- Immunodeficiency and Autoimmune Disorders
- Inflammasome and immune disorders
- Renal Diseases and Glomerulopathies
- Vasculitis and related conditions
- Interstitial Lung Diseases and Idiopathic Pulmonary Fibrosis
- Dermatological and Skeletal Disorders
- Eosinophilic Disorders and Syndromes
- Rheumatoid Arthritis Research and Therapies
- Inflammatory Myopathies and Dermatomyositis
- Kawasaki Disease and Coronary Complications
- Family and Disability Support Research
- Acute Lymphoblastic Leukemia research
- Tuberculosis Research and Epidemiology
- Inflammatory Bowel Disease
- Diabetes and associated disorders
- Otitis Media and Relapsing Polychondritis
- Streptococcal Infections and Treatments
- Immune Cell Function and Interaction
- Neurogenetic and Muscular Disorders Research
- Medical Imaging and Pathology Studies
- Atherosclerosis and Cardiovascular Diseases
Hospital Universitário Pedro Ernesto
2014-2025
Universidade do Estado do Rio de Janeiro
2014-2024
Universidade Federal do Rio de Janeiro
2014-2024
Secretaria de Agricultura e Abastecimento
2024
Universidade Federal do Estado do Rio de Janeiro
2024
Istituto Giannina Gaslini
2009-2018
Cincinnati Children's Hospital Medical Center
2018
Sydney Children's Hospital
2018
University of Cincinnati
2018
Institut Universitaire de Gériatrie de Montréal
2016
<h3>Objectives</h3> To validate the previously proposed classification criteria for Henoch–Schönlein purpura (HSP), childhood polyarteritis nodosa (c-PAN), c-Wegener granulomatosis (c-WG) and c-Takayasu arteritis (c-TA). <h3>Methods</h3> Step 1: retrospective/prospective web-data collection children with HSP, c-PAN, c-WG c-TA age at diagnosis ≤18 years. 2: blinded by consensus panel of a representative sample 280 cases. 3: statistical (sensitivity, specificity, area under curve κ-agreement)...
Interleukin-1 is pivotal in the pathogenesis of systemic juvenile idiopathic arthritis (JIA). We assessed efficacy and safety canakinumab, a selective, fully human, anti–interleukin-1β monoclonal antibody, two trials.
Abstract Objective To investigate the long‐term outcome and prognostic factors of juvenile dermatomyositis (DM) through a multinational, multicenter study. Methods Patients consisted inception cohorts seen between 1980 2004 in 27 centers Europe Latin America. Predictor variables were sex, continent, ethnicity, onset year, age, type, manifestations, course disease duration, active duration. Outcomes muscle strength/endurance, continued activity, cumulative damage, cutaneous calcinosis,...
The purpose of this study was to obtain data on the association antiphospholipid antibodies with clinical manifestations in childhood and enable future studies determine impact treatment long-term outcome pediatric syndrome.A European registry extended internationally patients syndrome established as a collaborative project Antiphospholipid Antibodies Forum Lupus Working Group Pediatric Rheumatology Society. To be eligible for enrollment patient must meet preliminary criteria classification...
To determine the clinical and immunologic features of systemic sclerosis (SSc) in a large group children describe evolution disease compare it with adult form.Data on 153 patients juvenile SSc collected from 55 pediatric rheumatology centers Europe, Asia, South North America were analyzed. Demographic, clinical, characteristics at onset, diagnosis, during course evaluated.Raynaud's phenomenon was most frequent symptom, followed by skin induration approximately 75% patients. Musculoskeletal...
Objective To compare the capacity of 2004 diagnostic guidelines for hemophagocytic lymphohistiocytosis (HLH‐2004) with preliminary systemic juvenile idiopathic arthritis (JIA)–associated macrophage activation syndrome (MAS) to discriminate MAS complicating JIA from 2 potentially confusable conditions, represented by active without and infection. Methods International pediatric rheumatologists hemato‐oncologists were asked retrospectively collect clinical information patients JIA–associated...
This report aims to determine the safety, pharmacokinetics (PK) and efficacy of subcutaneous golimumab in active polyarticular-course juvenile idiopathic arthritis (polyJIA).In this three-part randomised double-blinded placebo-controlled withdrawal trial, all patients received open-label (30 mg/m2 body surface area; maximum: 50 mg/dose) every 4 weeks together with weekly methotrexate during Part 1 (weeks 0-16). Patients at least 30% improvement per American College Rheumatology Criteria for...
Abstract Objective To develop and validate a new short simple measure of physical function in children with juvenile idiopathic arthritis (JIA). Methods The Juvenile Arthritis Functionality Scale (JAFS) is 15‐item questionnaire that explores 3 body areas (lower limbs, hand/wrist, upper segment). Validation the Italian version instrument was accomplished by evaluating 211 consecutive JIA patients ages 2.2–18 years. instrument's feasibility, face content validity, construct discriminative...
To determine cutoff values for defining the state of high disease activity (HDA) in juvenile idiopathic arthritis (JIA) using Juvenile Arthritis Disease Activity Score (JADAS).For selection values, data from a clinical database including 609 patients were used. Optimal determined against external criteria by calculating 25th and 10th centile cumulative score distribution through receiver operating characteristic curve analysis. External based on therapeutic decision made attending doctor....
To date there are no specific classification criteria for childhood-onset systemic lupus erythematosus (cSLE). This study aims to compare the performance among American College of Rheumatology (ACR) 1997, Systemic Lupus International Collaborating Clinics (SLICC) and new European League Against Rheumatism (EULAR)/ACR criteria, in a cSLE cohort. We conducted medical chart review cases controls with defined rheumatic diseases, both ANA positive, establish each ACR1997, SLICC EULAR/ACR...
To develop a composite disease activity score for systemic JIA (sJIA) and to provide preliminary evidence of its validity.The Juvenile Arthritis Disease Activity Score (sJADAS) was constructed by adding the four items original JADAS fifth item that aimed quantify features. Validation analyses were conducted on patients with definite or probable/possible sJIA enrolled at first visit time flare, who had active manifestations, which should include fever. Patients reassessed 2 weeks 3 months...
Abstract Objective To validate and promulgate a core set of outcome measures for the evaluation response to treatment in patients with juvenile systemic lupus erythematosus (SLE). Methods In 2001, preliminary consensus‐derived evaluating therapy SLE was established. present study, validated through an evidence‐based, large‐scale data collection process that led enrollment 557 from 39 different countries. Consecutive active disease were assessed at baseline after 6 months. The validation...
Abstract Objective To assess health‐related quality of life (HRQOL) in abatacept‐treated children/adolescents with juvenile idiopathic arthritis (JIA). Methods In this phase III, double‐blind, placebo‐controlled trial, subjects active polyarticular course JIA and an inadequate response/intolerance to ≥1 disease‐modifying antirheumatic drug (including biologics) received abatacept 10 mg/kg plus methotrexate (MTX) during the 4‐month open‐label period (period A). Subjects achieving American...
Mutations in genes coding for proteasome subunits and/or assembly helpers typically cause recurring autoinflammation referred to as chronic atypical neutrophilic dermatosis with lipodystrophy and elevated temperatures (CANDLE) or proteasome-associated autoinflammatory syndrome (PRAAS). Patients CANDLE/PRAAS present mostly chronically type I interferon scores that emerge a consequence of increased proteotoxic stress by mechanisms are not fully understood. Here, we report on five unrelated...
<h3>Objectives</h3> To determine whether baseline demographic, clinical, articular and laboratory variables predict methotrexate (MTX) poor response in polyarticular-course juvenile idiopathic arthritis. <h3>Methods</h3> Patients newly treated for 6 months with MTX enrolled the Paediatric Rheumatology International Trials Organization (PRINTO) trial. Bivariate logistic regression analyses were used to identify predictors of according American College pediatric (ACR-ped) 30 70 criteria....
Objective To describe the frequency and types of disease damage occurring with childhood‐onset systemic lupus erythematosus (SLE) as measured by 41‐item Systemic Lupus International Collaborating Clinics/American College Rheumatology Damage Index (SDI), to assess SDI's ability reflect severity. Methods Information for SDI was prospectively collected from 1,048 SLE patients. For a subset 559 patients, physician‐rated severity visual analog scale (MD VAS damage) also available. Frequency items...
To derive a list of opportunistic infections (OI) through the analysis juvenile idiopathic arthritis (JIA) patients in Pharmachild registry by an independent Safety Adjudication Committee (SAC). The SAC (3 pediatric rheumatologists and 2 infectious disease specialists) elaborated approved consensus provisional OI for use JIA. Through 5 step-procedure, all severe serious infections, classified as per MedDRA dictionary retrieved registry, were evaluated answering six questions adjudicated with...