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Ann Rheum Dis ; 78(10): 1405-1411, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31278138


OBJECTIVES: To describe the clinical characteristics, treatment response and genetic findings in a large cohort of patients with undefined systemic autoinflammatory diseases (SAIDs). METHODS: Clinical and genetic data from patients with undefined SAIDs were extracted from the Eurofever registry, an international web-based registry that retrospectively collects clinical information on patients with autoinflammatory diseases. RESULTS: This study included 187 patients. Seven patients had a chronic disease course, 180 patients had a recurrent disease course. The median age at disease onset was 4.3 years. Patients had a median of 12 episodes per year, with a median duration of 4 days. Most commonly reported symptoms were arthralgia (n=113), myalgia (n=86), abdominal pain (n=89), fatigue (n=111), malaise (n=104) and mucocutaneous manifestations (n=128). In 24 patients, relatives were affected as well. In 15 patients, genetic variants were found in autoinflammatory genes. Patients with genetic variants more often had affected relatives compared with patients without genetic variants (p=0.005). Most patients responded well to non-steroidal anti-inflammatory drugs (NSAIDs), corticosteroids, colchicine and anakinra. Complete remission was rarely achieved with NSAIDs alone. Notable patterns were found in patients with distinctive symptoms. Patients with pericarditis (n=11) were older at disease onset (33.8 years) and had fewer episodes per year (3.0/year) compared with other patients. Patients with an intellectual impairment (n=8) were younger at disease onset (2.2 years) and often had relatives affected (28.6%). CONCLUSION: This study describes the clinical characteristics of a large cohort of patients with undefined SAIDs. Among these, patients with pericarditis and intellectual impairment appear to comprise distinct subsets.

Arthritis Res Ther ; 20(1): 285, 2018 12 27.
Artigo em Inglês | MEDLINE | ID: mdl-30587248


BACKGROUND: The availability of methotrexate and the introduction of multiple biological agents have revolutionized the treatment of juvenile idiopathic arthritis (JIA). Several international and national drug registries have been implemented to accurately monitor the long-term safety/efficacy of these agents. This report aims to present the combined data coming from Pharmachild/PRINTO registry and the national registries from Germany (BiKeR) and Sweden. METHODS: Descriptive statistics was used for demographic, clinical data, drug exposure, adverse events (AEs) and events of special interest (ESIs). For the Swedish register, AE data were not available. RESULTS: Data from a total of 15,284 patients were reported: 8274 (54%) from the Pharmachild registry and 3990 (26%) and 3020 (20%) from the German and the Swedish registries, respectively. Pharmachild children showed a younger age (median of 5.4 versus 7.6 years) at JIA onset and shorter disease duration at last available visit (5.3 versus 6.1-6.8) when compared with the other registries. The most frequent JIA category was the rheumatoid factor-negative polyarthritis (range of 24.6-29.9%). Methotrexate (61-84%) and etanercept (24%-61.8%) were the most frequently used synthetic and biologic disease-modifying anti-rheumatic drugs (DMARDs), respectively. There was a wide variability in glucocorticoid use (16.7-42.1%). Serious AEs were present in 572 (6.9%) patients in Pharmachild versus 297 (7.4%) in BiKeR. Infection and infestations were the most frequent AEs (29.4-30.1%) followed by gastrointestinal disorders (11.5-19.6%). The most frequent ESIs were infections (75.3-89%). CONCLUSIONS: This article is the first attempt to present a very large sample of data on JIA patients from different national and international registries and represents the first proposal for data merging as the most powerful tool for future analysis of safety and effectiveness of immunosuppressive therapies in JIA. REGISTRY REGISTRATION: The Pharmachild registry is registered at ( NCT01399281 ) and at the European Network of Centres for Pharmacoepidemiology and Pharmacovigilance (ENCePP) ( ). The BiKeR registry is registered at ENCePP ( ).

Artrite Juvenil/tratamento farmacológico , Produtos Biológicos/uso terapêutico , Farmacovigilância , Sistema de Registros/estatística & dados numéricos , Medicamentos Sintéticos/uso terapêutico , Adolescente , Antirreumáticos/efeitos adversos , Antirreumáticos/uso terapêutico , Artrite Juvenil/diagnóstico , Produtos Biológicos/efeitos adversos , Criança , Pré-Escolar , Monitoramento de Medicamentos , Etanercepte/efeitos adversos , Etanercepte/uso terapêutico , Feminino , Humanos , Masculino , Metotrexato/efeitos adversos , Metotrexato/uso terapêutico , Estudos Prospectivos , Estudos Retrospectivos , Medicamentos Sintéticos/efeitos adversos , Resultado do Tratamento
Stomatologija ; 18(2): 51-60, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27649720


INTRODUCTION: Patients with juvenile idiopathic arthritis (JIA) have a high risk of temporomandibular joint (TMJ) involvement. Lesions in the TMJ appear early in the course of this disease. Evaluating the structure of the TMJ in JIA patients using cone beam computed tomography (CBCT) provides an understanding of the typical radiologic features of morphological change in TMJs of JIA patients. This study aims to report these features as seen in CBCT and thus comparing them with the features observed in a control group within the same age group and in females and males. MATERIALS AND METHODS: Cross-sectional observational study whereby CBCTs of 65 (130 joints) patients with a confirmed JIA diagnosis and 30 (60 joints) control group - patients without JIA upto the age of 17. Structural radiologic features of the joint's hard tissues were assessed according to the research diagnostic criteria for temporomandibular disorders as developed by Ahmad et al. RESULTS: The radiologic features of the osseous structures of the TMJ occurred asymmetrically between the right and left sides when compared in the JIA and control groups. The most prevalent feature in the JIA group is condyle surface flattening for both sides. Condyle surface erosion and osteophyte were also frequent and occurred with high statistical significance in both males and females. CONCLUSIONS: TMJ destruction features observed in CBCT images were prevalent in the JIA group and occurred infrequently in the control group.

Artrite Juvenil/diagnóstico por imagem , Tomografia Computadorizada de Feixe Cônico , Transtornos da Articulação Temporomandibular/diagnóstico por imagem , Articulação Temporomandibular/diagnóstico por imagem , Adolescente , Artrite Juvenil/patologia , Criança , Estudos Transversais , Feminino , Humanos , Masculino , Articulação Temporomandibular/patologia , Transtornos da Articulação Temporomandibular/patologia
Pediatr Rheumatol Online J ; 14(1): 24, 2016 Apr 19.
Artigo em Inglês | MEDLINE | ID: mdl-27095316


BACKGROUND: Temporomandibular joint (TMJ) arthritis is seen very often (38-87 %) in children with juvenile idiopathic arthritis (JIA). With contrast enhanced magnetic resonance imaging (MRI) we can detect more cases of TMJ arthritis than ever before. Previous studies show that HLA II class alleles may have protective or risk importance in JIA subtypes. Our objective is to identify HLA II class alleles of risk and protection in JIA patients with TMJ arthritis. METHODS: During the period from 2010 to 2015 MRI for TMJ was performed in 85 JIA patients who were genotyped for HLA- DRB1; DQB1 and DQA1 using RT-PCR with sequence-specific primers. As a control group, data of 100 individuals were taken from the genetic bank of RSU Joint Laboratory of Clinical Immunology and Immunogenetics. Associations of DRB1; DQB1; DQA1 alleles in patients were examined individually using the χ (2) test. P-value (<0.05) and odds ratio were calculated using EPI INFO 6.0 software. RESULTS: Out of 85 JIA patients with mean age of 13.7 ± 3.0 years (range 6.9-17.9 years), 59 (69 %) were girls and 26 (31 %) were boys. The mean duration of the disease was 3.07 ± 2.35 years (range 0.2-11.0 year). JIA subtypes were as follows: seronegative polyarthritis 51 (60 %), seropositive polyarthritis 6(7 %), oligoarthritis extended 7(8 %), oligoarthritis persistent 2 (2 %) arthritis with enthesitis 14 (17 %), undifferentiated 3 (4 %) and 2 (2 %) systemic arthritis. Two groups where separated after TMJ MRI exam: first with at least two signs of active inflammation and/or any structural damage (n = 62); second with no pathologic signs or with slight contrast enhancement (n = 23). We discovered that there are risk alleles that are found in all JIA patient's groups (MRI positive and negative groups) versus controls such as DRB1*07:01, DQB1*03:03; DQB1*05:01. Also some protective alleles as DRB1*18:01, DQB1*06:02-8 were found in overall JIA group. Alleles DRB1*12:01, DQB1*03:01; DQA1*05:01 were found to be protective for TMJ arthrits. CONCLUSION: In our study there were no convincing risk alleles, but there are alleles that probably are protective for TMJ arthritis like DRB1*12:01, DQB1*03:01; DQA1*05:01.

Artrite Juvenil/genética , DNA/genética , Predisposição Genética para Doença , Antígenos de Histocompatibilidade Classe II/genética , Mutação , Articulação Temporomandibular , Adolescente , Alelos , Artrite Juvenil/diagnóstico , Artrite Juvenil/imunologia , Criança , Feminino , Genótipo , Antígenos de Histocompatibilidade Classe II/imunologia , Humanos , Imagem por Ressonância Magnética , Masculino , Razão de Chances , Reação em Cadeia da Polimerase em Tempo Real , Estudos Retrospectivos
Ann Rheum Dis ; 73(6): 1114-22, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23696632


OBJECTIVE: To investigate the efficacy and safety of etanercept (ETN) in paediatric subjects with extended oligoarticular juvenile idiopathic arthritis (eoJIA), enthesitis-related arthritis (ERA), or psoriatic arthritis (PsA). METHODS: CLIPPER is an ongoing, Phase 3b, open-label, multicentre study; the 12-week (Part 1) data are reported here. Subjects with eoJIA (2-17 years), ERA (12-17 years), or PsA (12-17 years) received ETN 0.8 mg/kg once weekly (maximum 50 mg). Primary endpoint was the percentage of subjects achieving JIA American College of Rheumatology (ACR) 30 criteria at week 12; secondary outcomes included JIA ACR 50/70/90 and inactive disease. RESULTS: 122/127 (96.1%) subjects completed the study (mean age 11.7 years). JIA ACR 30 (95% CI) was achieved by 88.6% (81.6% to 93.6%) of subjects overall; 89.7% (78.8% to 96.1%) with eoJIA, 83.3% (67.2% to 93.6%) with ERA and 93.1% (77.2% to 99.2%) with PsA. For eoJIA, ERA, or PsA categories, the ORs of ETN vs the historical placebo data were 26.2, 15.1 and 40.7, respectively. Overall JIA ACR 50, 70, 90 and inactive disease were achieved by 81.1, 61.5, 29.8 and 12.1%, respectively. Treatment-emergent adverse events (AEs), infections, and serious AEs, were reported in 45 (35.4%), 58 (45.7%), and 4 (3.1%), subjects, respectively. Serious AEs were one case each of abdominal pain, bronchopneumonia, gastroenteritis and pyelocystitis. One subject reported herpes zoster and another varicella. No differences in safety were observed across the JIA categories. CONCLUSIONS: ETN treatment for 12 weeks was effective and well tolerated in paediatric subjects with eoJIA, ERA and PsA, with no unexpected safety findings.

Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Artrite Psoriásica/tratamento farmacológico , Imunoglobulina G/uso terapêutico , Receptores do Fator de Necrose Tumoral/uso terapêutico , Adolescente , Artrite Juvenil/fisiopatologia , Artrite Psoriásica/fisiopatologia , Criança , Pré-Escolar , Etanercepte , Feminino , Humanos , Masculino , Índice de Gravidade de Doença , Resultado do Tratamento