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1.
Eur Radiol ; 28(9): 3963-3976, 2018 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-29582130

RESUMO

BACKGROUND: Juvenile idiopathic arthritis (JIA) can cause structural damage. However, data on conventional radiography (CR) in JIA are scant. OBJECTIVE: To provide pragmatic guidelines on CR in each non-systemic JIA subtype. METHODS: A multidisciplinary task force of 16 French experts (rheumatologists, paediatricians, radiologists and one patient representative) formulated research questions on CR assessments in each non-systemic JIA subtype. A systematic literature review was conducted to identify studies providing detailed information on structural joint damage. Recommendations, based on the evidence found, were evaluated using two Delphi rounds and a review by an independent committee. RESULTS: 74 original articles were included. The task force developed four principles and 31 recommendations with grades ranging from B to D. The experts felt strongly that patients should be selected for CR based on the risk of structural damage, with routine CR of the hands and feet in rheumatoid factor-positive polyarticular JIA but not in oligoarticular non-extensive JIA. CONCLUSION: These first pragmatic recommendations on CR in JIA rely chiefly on expert opinion, given the dearth of scientific evidence. CR deserves to be viewed as a valuable tool in many situations in patients with JIA. KEY POINTS: • CR is a valuable imaging technique in selected indications. • CR is routinely recommended for peripheral joints, when damage risk is high. • CR is recommended according to the damage risk, depending on JIA subtype. • CR is not the first-line technique for imaging of the axial skeleton.


Assuntos
Artrite Juvenil/diagnóstico por imagem , Adolescente , Artrite Juvenil/classificação , Criança , Feminino , Humanos , Masculino , Radiografia
2.
Clin Exp Rheumatol ; 35(4): 638-646, 2017.
Artigo em Inglês | MEDLINE | ID: mdl-28516872

RESUMO

OBJECTIVES: Anti-TNFα agents are indicated in selected patients with rheumatoid arthritis (RA) who respond inadequately to methotrexate and particularly when glucocorticoids are mandatory. We evaluated whether a glucocorticoid-sparing effect occurred during the first year of anti-TNF-α therapy. METHODS: Between 2007 and 2009, the French multicentre, longitudinal, prospective, observational, population-based CORPUS cohort included biologic-naive patients with inflammatory joint disease. Patients with active RA treated with glucocorticoids were included. Patients who received at least one anti-TNFα injection during follow-up were compared to anti-TNF-α non-users. RESULTS: Among the 205 patients, 76.1% were women, mean disease duration was 7.7±8.3 years, mean DAS28 was 5.2±1.3, mean follow-up was 13.1±2.8 months, and mean prednisone dose was 9.9±9.6 mg/day. The 75 (36.6%) anti-TNF-α recipients were younger, had a longer RA duration, more often tested positive for rheumatoid factor and anti-citrullinated peptide antibody, more often received previous DMARDs, received a higher methotrexate dosage, had fewer intra-articular glucocorticoid injections at baseline and were more often followed by hospital practitioners than non-recipients. Mean prednisone dosage decreased from 11.8±12.7 to 5.9±9.7 mg/day in recipients and from 8.7±7.1 to 5.0±4.4 mg/day in non-recipients. Prednisone was stopped more often among recipients (21/59, 35.6%) than among non-recipients (16/94, 17.0%) (p=0.01). By multivariate analysis, factors independently associated with lower prednisone requirements were baseline daily prednisone dosage, a CRP >10 mg/l and not to be followed by an office-based practitioner. CONCLUSIONS: This study showed a significantly higher glucocorticoid discontinuation rate among anti-TNF-α recipients than among non-recipients. However, the glucocorticoid-sparing effect was small and not observed by multivariate analysis.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Reumatoide/tratamento farmacológico , Glucocorticoides/uso terapêutico , Metotrexato/uso terapêutico , Prednisona/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adulto , Idoso , Artrite Reumatoide/imunologia , Autoanticorpos/imunologia , Proteína C-Reativa/imunologia , Estudos de Coortes , Quimioterapia Combinada , Feminino , Humanos , Injeções Intra-Articulares , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Análise Multivariada , Peptídeos Cíclicos/imunologia , Estudos Prospectivos , Fator Reumatoide/imunologia
3.
J Inherit Metab Dis ; 38(5): 791-6, 2015 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-25860819

RESUMO

Alkaptonuria (AKU) is caused by deficiency of the enzyme homogentisate 1,2 dioxygenase. It results in an accumulation of homogentisate which oxidizes spontaneously to benzoquinone acetate, a highly oxidant compound, which polymerises to a melanin-like structure, in a process called ochronosis. Asymptomatic during childhood, this accumulation will lead from the second decade of life to a progressive and severe spondylo-arthopathy, associated with multisystem involvement: osteoporosis/fractures, stones (renal, prostatic, gall bladder, salivary glands), ruptures of tendons/muscle/ligaments, renal failure and aortic valve disease. The pathophysiological mechanisms of AKU remain poorly understood, but recent advances lead us to reconsider the treatment strategy in AKU patients. Besides the supporting therapies (pain killers, anti-inflammatory drugs, physiotherapy, joints replacements and others), specific therapies have been considered (anti-oxidant, low protein diet, nitisinone), but clinical studies have failed to prove efficiency on the rheumatological lesions of the disease. Here we propose a treatment strategy for children and adults with AKU, based on a review of the latest findings on AKU and lessons from other aminoacipathies, especially tyrosinemias.


Assuntos
Alcaptonúria/tratamento farmacológico , Adulto , Fatores Etários , Alcaptonúria/dietoterapia , Alcaptonúria/epidemiologia , Antioxidantes/uso terapêutico , Criança , Cicloexanonas/uso terapêutico , Humanos , Nitrobenzoatos/uso terapêutico , Ocronose/dietoterapia , Ocronose/tratamento farmacológico , Ocronose/epidemiologia , Fenilalanina/administração & dosagem , Tirosina/administração & dosagem
4.
Clin Exp Rheumatol ; 33(5): 602-10, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26315585

RESUMO

OBJECTIVES: Limited information is available about the characteristics of patients with active inflammatory rheumatic diseases who start TNF-α antagonist therapy. Our objective was to assess TNF-α antagonist prescription patterns in this context in France. METHODS: Between 2007 and 2009, 102 rheumatologists, internists, and pediatricians in French university hospitals and private practice prospectively recruited biologics-naïve patients with active rheumatoid arthritis (RA) (DAS28>3.2 despite methotrexate therapy), spondyloarthritis (SA) (BASDAI≥4 despite non-steroidal anti-inflammatory drug [NSAID] use), and juvenile idiopathic arthritis (JIA) (unresponsive to methotrexate). Patients were monitored prospectively for 1 year. RESULTS: Of the 543 RA, 287 SA, and 53 JIA patients included in the study, 382 RA, 171 SA, and 28 JIA patients had complete follow-up data available after 1 year. Among these patients, 110/382 (28.8%) with RA, 81/171 (47.4%) with SA, and 26/28 (92.9%) with JIA received at least one TNF-α antagonist dose during the 1-year follow-up. The main physician-reported reason for not starting TNF-α antagonists in patients with RA or SA was low disease activity (72% for RA and 67% for SA); absence of TNF-α antagonist therapy was due to patient refusal in only 10% and to contraindications in 6% to 7% of cases. CONCLUSIONS: In France, TNF-α antagonists, which are fully reimbursed by the national health insurance system, were used almost routinely in JIA patients unresponsive to methotrexate and were given to about half the SA patients with BASDAI≥4 despite NSAID use and a third of RA patients with DAS28>3.2 despite methotrexate therapy.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Artrite Reumatoide/tratamento farmacológico , Fatores Imunológicos/uso terapêutico , Seleção de Pacientes , Padrões de Prática Médica/tendências , Espondiloartropatias/tratamento farmacológico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adolescente , Adulto , Idoso , Artrite Juvenil/diagnóstico , Artrite Juvenil/imunologia , Artrite Reumatoide/diagnóstico , Artrite Reumatoide/imunologia , Criança , Contraindicações , Prescrições de Medicamentos , Revisão de Uso de Medicamentos , Feminino , França , Humanos , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Estudos Prospectivos , Índice de Gravidade de Doença , Espondiloartropatias/diagnóstico , Espondiloartropatias/imunologia , Fatores de Tempo , Resultado do Tratamento , Recusa do Paciente ao Tratamento , Fator de Necrose Tumoral alfa/imunologia , Adulto Jovem
5.
Ann Rheum Dis ; 73(6): 1114-22, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-23696632

RESUMO

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.


Assuntos
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
6.
Rheumatology (Oxford) ; 52(2): 267-75, 2013 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-22513153

RESUMO

OBJECTIVES: Radiological cervical spine involvement in JIA has already been assessed with a large range of prevalence (5-80%), but most studies were performed a long time ago, in symptomatic JIA and without differentiating subsets of JIA. We set out to describe structural cervical spine involvement in young adults with polyarticular JIA (pJIA) regardless of the cervical symptoms and to compare lesions with those observed in adult RA. METHODS: All consecutive pJIAs followed in a transition programme were included. Standard radiographs of the cervical spine, hands, feet and hip were analysed by two independent radiologists blinded to the diagnosis. An RA control group (<55 years), matched for sex and disease duration, was recruited. RESULTS: Fifty-seven pJIA and 58 RA patients were included. Radiographs showed cervical lesions in 65% of pJIA and 67% of RA patients. In total, 51% of pJIA with radiographic abnormalities had no clinical symptoms. In pJIA, the most frequent structural lesions were anterior atlantoaxial subluxation (33%), erosion of the odontoid process (19%), C1-C2 arthritis (17%) and apophyseal joint arthritis (16%). Cervical lesions in pJIA were similar to those in RA except for ankylosis and hypotrophia (P < 0.05). The presence of cervical lesions correlated with a more severe disease. CONCLUSION: Structural cervical spine involvement is common in pJIA persisting into adulthood, frequently asymptomatic and associated with a more severe disease. We suggest that radiographic assessment of the cervical spine should be done systematically at onset of the disease and regularly during its course regardless of clinical symptoms.


Assuntos
Artrite Juvenil/diagnóstico por imagem , Vértebras Cervicais/diagnóstico por imagem , Doenças da Coluna Vertebral/diagnóstico por imagem , Adolescente , Adulto , Artrite Juvenil/sangue , Artrite Reumatoide/sangue , Artrite Reumatoide/diagnóstico por imagem , Sedimentação Sanguínea , Proteína C-Reativa/metabolismo , Estudos de Casos e Controles , Criança , Estudos de Coortes , Estudos Transversais , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Prevalência , Radiografia , Índice de Gravidade de Doença , Doenças da Coluna Vertebral/sangue , Adulto Jovem
7.
Pediatr Radiol ; 43(3): 355-75, 2013 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-23263195

RESUMO

BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is an autoinflammatory disorder that is currently diagnosed based on clinical, radiologic, pathological and longitudinal findings. OBJECTIVE: To provide detailed descriptions of CRMO lesion patterns seen on radiographs and MRI and to suggest clinical use of whole-body MRI and propose noninvasive diagnostic strategy. MATERIALS AND METHODS: Retrospective longitudinal study (1989-2010) of 31 children (22 girls, 9 boys) diagnosed with CRMO. Imaging data were evaluated by two pediatric radiologists. RESULTS: Mean age at diagnosis was 11 years (3-17). A total of 108 lesions were investigated. The most common sites were the long bone metaphyses (56 lesions in 24 children) especially femoral and tibial (20/24); pelvis (10/31); spine (9/31); clavicle (6/31) and mandible (3/31). In long bones, the radiologic appearance was normal (22/56), mixed lytic and sclerotic (20/56), sclerotic (8/56) or lytic (6/56) often juxtaphyseal (36/56), with hyperostosis or periosteal thickening (10/56). Vertebral involvement was often multifocal (6/9). Medullary edema was seen on MRI (42) with epiphyseal (23/42) or soft-tissue (22/42) inflammation and juxtaphyseal nodule-like appearance (7/42). Whole-body MRI (15/31) was key in detecting subclinical lesions. CONCLUSION: CRMO is a polymorphous disorder in which whole-body MRI is extremely useful for showing subclinical edema. Vertebral collapse requires long-term monitoring.


Assuntos
Imageamento por Ressonância Magnética/métodos , Osteomielite/patologia , Imagem Corporal Total/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Estudos Longitudinais , Masculino , Recidiva , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade
8.
Joint Bone Spine ; 90(2): 105501, 2023 03.
Artigo em Inglês | MEDLINE | ID: mdl-36574572

RESUMO

OBJECTIVES: Polyarticular juvenile idiopathic arthritis (pJIA) is a subset of juvenile idiopathic arthritis (JIA), divided into two subtypes according to the presence of rheumatoid factor: pJIA without rheumatoid factor (pJIA RF-) and pJIA with positive rheumatoid factor (pJIA RF+), this latter is characterised with more structural damage. Anti-citrullinated peptide antibodies (ACPA) are often associated with RF. The respective performance of ACPA versus RF in structural outcome in pJIA, and in particular in adulthood pJIA remains unknown. Therefore, the aim of this study was to determine whether ACPA could be of value to assess structural damage in pJIA persisting in adulthood. METHODS: Patients with pJIA and available data for ACPA, RF and X-ray were included retrospectively. Structural damage was assessed by two independent blinded investigators using Sharp Van Der Heijde scores. RESULTS: 56 pJIA adult patients were included: 62% (35/56) had pJIA RF+ and 38% (21/56) pJIA RF-. ACPA positivity in pJIA was significantly associated with presence of RF (96% vs 26%, P<0.001). RF positivity was significantly associated with higher Sharp van Der Heijde erosion and total scores (respectively P<0.01 and P<0.05). There were higher Sharp Van Der Heijde erosion, joint space narrowing and total scores in the pJIA ACPA+ subgroup than in the pJIA ACPA- subgroup, although there was no statistical significance. However, when adjusted on disease duration, pJIA ACPA+ patients had significantly higher erosion and total scores than pJIA ACPA- patients (P<0.05), and pJIA ACPA+ patients required more bDMARDs than pJIA ACPA- patients (P<0.05). Moreover, pJIA patients with high Sharp van Der Heijde joint space narrowing and total scores had significantly higher ACPA levels (P<0.01). A correlation was identified between ACPA levels and Sharp van Der Heijde total score (r=0.54, P<0.05). In the pJIA RF+ subgroup the presence of ACPA was associated with additional structural damage compared to no ACPA: sharp Van Der Heijde erosion, joint space narrowing and total scores were higher in the pJIA RF+ ACPA+ subgroup than in the pJIA RF+ ACPA- subgroup although these results did not reach significance. CONCLUSION: Our results suggest that pJIA RF+ ACPA+ adult patients may have a more severe articular phenotype than pJIA RF+ ACPA- patients. ACPA could bring an additional value to RF for pJIA patients regarding structural damage. Altogether our results show that RF and ACPA are associated with structural damage measured by Sharp Van Der Heijde score in pJIA persisting in adulthood.


Assuntos
Artrite Juvenil , Artrite Reumatoide , Humanos , Fator Reumatoide , Anticorpos Antiproteína Citrulinada , Estudos Retrospectivos , Medição de Risco , Autoanticorpos
9.
Ann Rheum Dis ; 71(4): 511-7, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21998114

RESUMO

OBJECTIVE: To obtain longitudinal data on growth/puberty in a large-scale, multi-national prospective cohort of juvenile systemic lupus erythematosus (SLE). METHODS: Data from 331/557 (59.4%) patients ≤18 years old with juvenile SLE in active phase, with anthropometric data available at four follow-up visits, were studied. RESULTS: There was a significant reduction in parent-adjusted height z score with time in females and males (p<0.0001), with a significant gender difference (p<0.0001) and with male height being most affected. Median body mass index z score peaked at 6 months and was still significantly above baseline after 26 months (p<0.01), with no gender difference. Standardised height reduction was inversely related to age at onset. Females with onset age <12 years had a median parent-adjusted height z score of -0.87 with no catch-up growth. At the end of the study, growth failure was seen in 14.7% of the females and 24.5% of the males. Height deflection (less than -0.25/year) was found in 20.7% of the females and 45.5% of the males. Delayed pubertal onset was seen in 15.3% and 24% of the females and males, respectively, and delayed/absent menarche was seen in 21.9%, while 36.1% of the females and 44% of the males had some degree of delayed pubertal development. Growth failure baseline determinants were previous growth failure (OR: 56.6), age at first visit ≤13.4 years (OR: 4.2) and cumulative steroid dose >426 mg/kg (OR: 3.6). CONCLUSIONS: The children at risk of having a negative effect on height and pubertal development are prepubertal and peripubertal children treated with >400 mg/kg cumulative dose of corticosteroids.


Assuntos
Transtornos do Crescimento/etiologia , Lúpus Eritematoso Sistêmico/complicações , Puberdade Tardia/etiologia , Adolescente , Idade de Início , Antropometria/métodos , Estatura/fisiologia , Índice de Massa Corporal , Criança , Esquema de Medicação , Feminino , Glucocorticoides/administração & dosagem , Glucocorticoides/efeitos adversos , Crescimento/fisiologia , Transtornos do Crescimento/fisiopatologia , Humanos , Estudos Longitudinais , Lúpus Eritematoso Sistêmico/tratamento farmacológico , Lúpus Eritematoso Sistêmico/fisiopatologia , Masculino , Estudos Prospectivos , Puberdade/fisiologia , Puberdade Tardia/fisiopatologia , Fatores Sexuais
10.
Front Med (Lausanne) ; 9: 1000167, 2022.
Artigo em Inglês | MEDLINE | ID: mdl-36388918

RESUMO

Objective: The frequency of vasculitis may be increased in patients with Familial Mediterranean Fever (FMF), according to several studies. Our aim was to assess the characteristics of French adult patients with both diseases. Methods: Patients with vasculitis were selected from patients followed for FMF in the French JIR-cohort. Results: Twenty-two patients were included [polyarteritis nodosa (PAN) n = 10, IgA vasculitis n = 8, unclassified vasculitis n = 2, granulomatosis with polyangiitis n = 1, and microscopic polyangiitis n = 1]. Pathogenic mutations in exon 10 were found in all 21 patients (96%) for which MEFV testing results were available, and 18 (82%) had two pathogenic mutations. Histology showed vasculitis in 59% of patients. Most patients with FMF-associated PAN were HBV-negative and had an inactive FMF before PAN onset, and 40% had a peri-renal or central nervous system bleeding. Most patients with FMF-associated IgA vasculitis had an active FMF before vasculitis onset, and 25% had digestive bleeding. Both patients with unclassified vasculitis had ischemic and/or hemorrhagic complications. Conclusion: This study confirms the predominance of PAN and IgA vasculitis in patients with FMF and the high frequency of bleeding in FMF-associated PAN. FMF should be considered in case of persistent symptoms and/or inflammatory syndrome despite vasculitis treatment in Mediterranean patients.

11.
Arthritis Rheum ; 62(6): 1792-802, 2010 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-20191582

RESUMO

OBJECTIVE: We previously documented that abatacept was effective and safe in patients with juvenile idiopathic arthritis (JIA) who had not previously achieved a satisfactory clinical response with disease-modifying antirheumatic drugs or tumor necrosis factor blockade. Here, we report results from the long-term extension (LTE) phase of that study. METHODS: This report describes the long-term, open-label extension phase of a double-blind, randomized, controlled withdrawal trial in 190 patients with JIA ages 6-17 years. Children were treated with 10 mg/kg abatacept administered intravenously every 4 weeks, with or without methotrexate. Efficacy results were based on data derived from the 153 patients who entered the open-label LTE phase and reflect >or=21 months (589 days) of treatment. Safety results include all available open-label data as of May 7, 2008. RESULTS: Of the 190 enrolled patients, 153 entered the LTE. By day 589, 90%, 88%, 75%, 57%, and 39% of patients treated with abatacept during the double-blind and LTE phases achieved responses according to the American College of Rheumatology (ACR) Pediatric 30 (Pedi 30), Pedi 50, Pedi 70, Pedi 90, and Pedi 100 criteria for improvement, respectively. Similar response rates were observed by day 589 among patients previously treated with placebo. Among patients who had not achieved an ACR Pedi 30 response at the end of the open-label lead-in phase and who proceeded directly into the LTE, 73%, 64%, 46%, 18%, and 5% achieved ACR Pedi 30, Pedi 50, Pedi 70, Pedi 90, and Pedi 100 responses, respectively, by day 589 of the LTE. No cases of tuberculosis and no malignancies were reported during the LTE. Pneumonia developed in 3 patients, and multiple sclerosis developed in 1 patient. CONCLUSION: Abatacept provided clinically significant and durable efficacy in patients with JIA, including those who did not initially achieve an ACR Pedi 30 response during the initial 4-month open-label lead-in phase.


Assuntos
Artrite Juvenil/tratamento farmacológico , Imunoconjugados/efeitos adversos , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Abatacepte , Adolescente , Antirreumáticos/efeitos adversos , Antirreumáticos/uso terapêutico , Criança , Método Duplo-Cego , Esquema de Medicação , Quimioterapia Combinada , Humanos , Imunoconjugados/uso terapêutico , Metotrexato/uso terapêutico , Índice de Gravidade de Doença , Resultado do Tratamento
12.
Arthritis Rheumatol ; 73(3): 530-541, 2021 03.
Artigo em Inglês | MEDLINE | ID: mdl-32951358

RESUMO

OBJECTIVE: To report the 2-year efficacy and safety of tocilizumab (TCZ) in patients with polyarticular-course juvenile idiopathic arthritis (JIA). METHODS: Patients ages 2-17 years with active polyarticular-course JIA, in whom treatment with methotrexate was unsuccessful, received 16 weeks of open-label intravenous TCZ in part 1 (once every 4 weeks: 8 mg/kg or 10 mg/kg for body weight [BW] <30 kg; 8 mg/kg for BW ≥30 kg). Assessments were based on the JIA-American College of Rheumatology (ACR) response (defined as percentage of improvement in ≥3 of the 6 JIA core response variables [CRVs]). Patients with at least a JIA-ACR30 response (defined as ≥30% improvement in ≥3 of the 6 JIA CRVs without worsening in >1 of the remaining JIA CRVs by >30%) at week 16 were randomly assigned (1:1) to receive TCZ or placebo in part 2. Patients remained in part 2 until either week 40 or the occurrence of JIA flare. Upon starting part 3, all patients received open-label TCZ. At week 104 of the study, efficacy was assessed using JIA-ACR50/70/90 response rates (defined as 50%, 70%, or 90% improvement, respectively), achievement of inactive disease, and the Juvenile Arthritis Disease Activity Score in 71 joints (JADAS-71). Safety was assessed in the all-exposure population per 100 patient-years of exposure. RESULTS: Overall, 188 patients entered part 1, 166 patients entered part 2, and 160 patients entered part 3. By week 104, among the 188 patients in the modified intent-to-treat group who received TCZ, JIA-ACR50/70/90 response rates were 80.3%/77.1%/59.6%, respectively, the median JADAS-71 score decreased from 3.6 at week 40 to 0.7 at week 104, 51.1% of patients had achieved inactive disease, and 31 of 66 patients who had been receiving glucocorticoids discontinued them. Adverse event (AE) and serious AE rates were 406.5 per 100 patient-years and 11.1 per 100 patient-years, respectively. The infection rate was 151.4 per 100 patient-years, and the serious infection rate was 5.2 per 100 patient-years. CONCLUSION: Patients treated with TCZ for polyarticular-course JIA showed high-level disease control for up to 2 years. The TCZ safety profile was consistent with that previously reported.


Assuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Infecções/epidemiologia , Adolescente , Artrite Juvenil/fisiopatologia , Bronquite/epidemiologia , Celulite (Flegmão)/epidemiologia , Doença Hepática Induzida por Substâncias e Drogas/epidemiologia , Varicela/epidemiologia , Criança , Pré-Escolar , Feminino , Glucocorticoides/administração & dosagem , Humanos , Masculino , Metotrexato/administração & dosagem , Medidas de Resultados Relatados pelo Paciente , Pneumonia/epidemiologia , Resultado do Tratamento
13.
J Pediatr ; 156(3): 484-9, 2010 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-19880136

RESUMO

OBJECTIVES: To study the pediatric presentation and evolution of relapsing polychondritis (RP), a rare inflammatory disease characterized by recurrent inflammation of cartilage. STUDY DESIGN: We retrospectively collected data from 10 patients observed in 3 French hospitals for relapsing polychondritis, with an age at onset <18 years. We also analyzed 37 cases of pediatric-onset RP from a systematic review. RESULTS: The mean age at first symptoms was 8.6 years, and the sex ratio was 6 male patients and 4 female patients. Children came to medical attention with joint pain, ocular inflammation, and chondritis. Outcomes included severe visual impairment, chronic destructive chondritis, and 1 death caused by aortic dilatation. Treatment mainly consisted of non-steroidal-anti-inflammatory drugs, corticosteroids, and immunosuppressants. Growth was normal in 7 examined patients. Systematic literature review also suggested a high number of tracheostomy in pediatric cases, but this was not confirmed in our series. CONCLUSION: RP in childhood shares the main clinical features of its adult counterpart, including destructive chondritis and systemic symptoms, but unlike adults, children frequently have a family history of autoimmunity and infrequently have other associated autoimmune diseases. RP can be fatal; close screening for complications is mandatory. Growth does not appear to be impaired by cartilage inflammation.


Assuntos
Policondrite Recidivante , Adolescente , Criança , Feminino , Humanos , Masculino , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico
14.
Semin Arthritis Rheum ; 50(4): 744-748, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32526594

RESUMO

OBJECTIVES: To assess the incidence rate and type of serious adverse events (SAE) in children with rheumatic inflammatory diseases treated with the interleukin 6 blocker tocilizumab (TCZ). METHODS: A retrospective review of all consecutive patients diagnosed with an inflammatory rheumatic disease and receiving at least one dose of TCZ was performed in two French tertiary pediatric rheumatology centers between 01/2007 and 06/2019. SAE were defined as a life-threatening event and/or an event requiring hospital admission, leading to permanent disability or treatment discontinuation. RESULTS: One hundred four children (64 female) were included. Most children suffered from systemic (n = 43) or polyarticular-course juvenile idiopathic arthritis (n = 43). Median age at TCZ start was 8.9 years (IQR 4.7 - 12.1), most children had received prednisone (81%), and/or a biologic agent (84%) prior to TCZ. Median TCZ treatment duration was 1.6 years (IQR 0.5 - 2.7), total TCZ exposure 215 patient years. Thirty-three SAE were observed in 26 (25%) children (SAE 15.3/100 patient years), mostly infections and infusion reactions. Children with SAE were significantly younger at disease onset (p = 0.034) and TCZ initiation (p = 0.016). Children experiencing infusion reactions were more likely to have systemic JIA or another autoinflammatory disease (p = 0.021), they all had active disease. At last follow up, 61 (59%) children remained on TCZ. CONCLUSION: In this cohort, SAE and most commonly serious infections were observed in a quarter of children. Severe infusion reactions were associated with persistently active autoinflammatory disease. Ongoing careful monitoring of TCZ-treated patients, especially young children with marked systemic inflammation is required.


Assuntos
Anticorpos Monoclonais Humanizados/administração & dosagem , Antirreumáticos/administração & dosagem , Artrite Juvenil/tratamento farmacológico , Anticorpos Monoclonais Humanizados/efeitos adversos , Antirreumáticos/efeitos adversos , Criança , Pré-Escolar , Feminino , Humanos , Infusões Intravenosas/efeitos adversos , Masculino , Estudos Retrospectivos
15.
Pediatr Rheumatol Online J ; 18(1): 1, 2020 Jan 02.
Artigo em Inglês | MEDLINE | ID: mdl-31898528

RESUMO

BACKGROUND: The aim of this study was to identify early clinical and laboratory features that distinguish acute lymphoblastic leukemia (ALL) from juvenile idiopathic arthritis (JIA) in children presenting with persistent bone or joint pain for at least 1 month. METHODS: We performed a multicenter case-control study and reviewed medical records of children who initially presented with bone or joint pain lasting for at least 1 month, all of whom were given a secondary diagnosis of JIA or ALL, in four French University Hospitals. Each patient with ALL was paired by age with two children with JIA. Logistic regression was used to compare clinical and laboratory data from the two groups. RESULTS: Forty-nine children with ALL and 98 with JIA were included. The single most important feature distinguishing ALL from JIA was the presence of hepatomegaly, splenomegaly or lymphadenopathy; at least one of these manifestations was present in 37 cases with ALL, but only in 2 controls with JIA, for an odds ratio (OR) of 154 [95%CI: 30-793] (regression coefficient: 5.0). If the presence of these findings is missed or disregarded, multivariate analyses showed that non-articular bone pain and/or general symptoms (asthenia, anorexia or weight loss) (regression coefficient: 4.8, OR 124 [95%CI: 11.4-236]), neutrophils < 2 × 109/L (regression coefficient: 3.9, OR 50 [95%CI: 4.3-58]), and platelets < 300 × 109/L (regression coefficient: 2.6, OR 14 [95%CI: 2.3-83.9]) were associated with the presence of ALL (area under the ROC curve: 0.96 [95%CI: 0.93-0.99]). CONCLUSIONS: Based on our findings we propose the following preliminary decision tree to be tested in prospective studies: in children presenting with at least 1 month of osteoarticular pain and no obvious ALL in peripheral smear, perform a bone marrow examination if hepatomegaly, splenomegaly or lymphadenopathy is present. If these manifestations are absent, perform a bone marrow examination if there is fever or elevated inflammatory markers associated with non-articular bone pain, general symptoms (asthenia, anorexia or weight loss), neutrophils < 2 × 109/L or platelets < 300 × 109/L.


Assuntos
Artralgia/etiologia , Artrite Juvenil/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Artrite Juvenil/diagnóstico , Artrite Juvenil/patologia , Estudos de Casos e Controles , Criança , Pré-Escolar , Árvores de Decisões , Diagnóstico Diferencial , Feminino , Hepatomegalia/etiologia , Humanos , Masculino , Leucemia-Linfoma Linfoblástico de Células Precursoras/diagnóstico , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia
16.
Pediatr Rheumatol Online J ; 17(1): 86, 2019 Dec 27.
Artigo em Inglês | MEDLINE | ID: mdl-31882011

RESUMO

BACKGROUND: Chronic musculoskeletal pain (MSP) is frequent in adolescents and has major medical and social consequences. In many cases, when no cause has been clearly established, this pain may be considered to be chronic idiopathic MSP. Our study seeks to identify general criteria for this type of pain through the experience of professionals from tertiary care centers with expertise in pediatric and adolescent chronic MSP. METHODS: Cross-sectional multicenter qualitative study. Semi-structured interviews of 25 professionals at a rheumatology reference center and in its network for pain management, including diverse specialists and professions. Interpretative Phenomenological Analysis is used to explore the data. RESULTS: This approach led us to identify 10 themes organized around three superordinate themes covering different stages of the diagnostic process: 1) the medical pain history up to the consultation at the reference center; 2) the professional's subjective feelings about the clinical presentation; 3) from the clinical examination to diagnosis and treatment of chronic idiopathic MSP. The main elements guiding this diagnosis do not come from the physical examination but from the medical history and the professionals' subjective feelings, that is, their clinical judgment. The professionals' impression of uneasiness and frustration, induced by patients and their parents, is of major importance. CONCLUSION: The principal elements guiding the diagnosis of chronic idiopathic MSP do not come primarily from the physical examination but rather from the pain history and the health professional's subjective feelings. Our results suggest that the concept of Juvenile Fibromyalgia (JFM) does not appear to cover all situations of chronic idiopathic MSP in adolescence. A constellation of non-organic criteria enables diagnosis of the latter; these criteria should be validated to avoid medical nomadism and multiple investigations and to shorten the interval until patients receive optimal pain management. CLINICAL TRIAL REGISTRATION: clinicaltrials.gov, NCT03171792, https://clinicaltrials.gov/ct2/show/NCT03171792?term=LACHAL&cntry=FR&city=paris&rank=1.


Assuntos
Dor Crônica/diagnóstico , Dor Musculoesquelética/diagnóstico , Manejo da Dor/métodos , Medição da Dor/métodos , Pesquisa Qualitativa , Adolescente , Adulto , Dor Crônica/terapia , Estudos Transversais , Feminino , Humanos , Masculino , Dor Musculoesquelética/terapia , Adulto Jovem
17.
Arthritis Res Ther ; 21(1): 125, 2019 05 23.
Artigo em Inglês | MEDLINE | ID: mdl-31122296

RESUMO

BACKGROUND: To describe the 6-year safety and efficacy of etanercept (ETN) in children with extended oligoarticular juvenile idiopathic arthritis (eoJIA), enthesitis-related arthritis (ERA), and psoriatic arthritis (PsA) METHODS: Patients who completed the 2-year, open-label, phase III CLinical Study In Pediatric Patients of Etanercept for Treatment of ERA, PsA, and Extended Oligoarthritis (CLIPPER) were allowed to enroll in its 8-year long-term extension (CLIPPER2). Children received ETN at a once-weekly dose of 0.8 mg/kg, up to a maximum dose of 50 mg/week. Efficacy assessments included the JIA core set of outcomes, the JIA American College of Rheumatology response criteria (JIA-ACR), and the Juvenile Arthritis Disease Activity Score (JADAS). Efficacy data are reported as responder analyses using a hybrid method for missing data imputation and as observed cases. Safety assessments included treatment-emergent adverse events (TEAEs). RESULTS: Out of 127 patients originally enrolled in CLIPPER, 109 (86%) entered CLIPPER2. After 6 years of trial participation (2 years in CLIPPER and 4 years in CLIPPER2), 41 (32%) patients were still taking ETN, 13 (11%) entered the treatment withdrawal phase after achieving low/inactive disease (of whom 7 had to restart ETN), 36 (28%) discontinued treatment for other reasons but are still being observed, and 37 (29%) discontinued treatment permanently. According to the hybrid imputation analysis, proportions of patients achieving JIA ACR90, JIA ACR100, and JADAS inactive disease after the initial 2 years of treatment were 58%, 48%, and 32%, respectively. After the additional 4 years, those proportions in patients who remained in the trial were 46%, 35%, and 24%. Most frequently reported TEAEs [n (%), events per 100 patient-years] were headache [28 (22%), 5.3], arthralgia [24 (19%), 4.6], and pyrexia [20 (16%), 3.8]. Number and frequency of TEAEs, excluding infections and injection site reactions, decreased over the 6-year period from 193 and 173.8, respectively, during year 1 to 37 and 61.3 during year 6. A single case of malignancy (Hodgkin's lymphoma) and no cases of active tuberculosis, demyelinating disorders, or deaths were reported. CONCLUSIONS: Open-label etanercept treatment for up to 6 years was safe, well tolerated, and effective in patients with eoJIA, ERA, and PsA. TRIAL REGISTRATION: ClinicalTrials.gov: CLIPPER, NCT00962741 , registered 20 August, 2009, CLIPPER2, NCT01421069 , registered 22 August, 2011.


Assuntos
Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Etanercepte/uso terapêutico , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Resultado do Tratamento
18.
Hum Vaccin Immunother ; 14(11): 2612-2617, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30230962

RESUMO

Objectives: Little information is available on the characteristics of elderly patients starting TNFα antagonist treatment for rheumatoid arthritis (RA). The objective of this work was to compare prescription patterns in RA patients younger vs. older than 75 years. Methods: Biologic-naive patients with active RA (DAS28 > 3.2) despite first-line therapy were included between 2007 and 2009 in the prospective, multicentre, longitudinal, observational, population-based CORPUS-RA cohort. TNFα antagonist users were defined as having received at least one TNFα antagonist during the first study year. The groups < 75 years and ≥ 75 years were compared regarding comorbidities, inflammation (CRP and ESR), disease activity (DAS28), disability (HAQ-DI), number of physician visits, and treatment. To verify the impact of the cut off, we also compared patients aged 70 years or more to patients younger than 70 years. Results: Of 543 RA patients, 382 had complete one-year follow-up data, including 114 TNFα antagonist users, 3 (6%) among the 49 patients aged 75 years or over and 111 (32%) of the 333 patients younger than 75 years (p < 0.01). Disease activity in the two age groups was similar at inclusion and after one year. Comorbidities and a history of auto-immunity were more common in the older group. Compared to their younger counterparts, the older patients received glucocorticoids more often (p = 0.003) and synthetic disease-modifying anti-rheumatic drugs less often (p = 0.01). Conclusion: TNFα antagonists are used less often and glucocorticoids more often in elderly patients with active RA compared to their younger counterparts. The fact that this study was performed in 2007-9 is a limitation in terms of relevance to today's patients and further studies should be conducted in new cohorts of active RA.

19.
Pediatr Rheumatol Online J ; 16(1): 21, 2018 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-29609643

RESUMO

BACKGROUND: Childhood-onset spondyloarthropathies usually start with enthesitis and peripheral arthritis. However, axial disease may develop afterward. Patients are most often classified, following revised (Edmonton 2011) ILAR criteria, as enthesitis-related arthritis, psoriatic arthritis, or unclassified juvenile idiopathic arthritis, particularly in cases of psoriasis in the patient or a first-degree relative. In adults, peripheral spondyloarthritis is classified by ASAS criteria. METHODS: We retrospectively studied patients with childhood-onset spondyloarthropathies followed for more than one year in our referral centre. We did not exclude patients with a personal or familial history of psoriasis. RESULTS: We included 114 patients followed between January 2008 and December 2015 for a median of 2.5 years (IQR = 2.3). Sixty-nine per-cent of patients fulfilled the revised ILAR classification criteria for enthesitis-related arthritis, and 92% the ASAS criteria for peripheral spondyolarthritis (p <  0.001). Axial disease and sacroiliitis were rare at disease onset. However, they appeared during follow-up in 63% and 47% of cases respectively, after a median disease duration of 2.6 (IC 95% [2.2-4.4]) and 5.3 years (IC 95% [4.1-7.7]), respectively. Multivariable analysis showed that familial history of spondyloarthritis was associated with the presence of sacroiliitis and active disease at the latest follow-up (OR = 3.61 [1.5-8.7], p <  0.01 and 2.98 [1.2-7.3], p = 0.02, respectively). CONCLUSION: Axial involvement developed in most patients within five years. Revised Edmonton criteria were less sensitive than ASAS criteria to classify patients as having childhood-onset spondyloarthropathies. The main risk factor for both sacroiliitis and persistent active disease was a familial history of spondyloarthritis.


Assuntos
Artrite Juvenil/diagnóstico , Espondilartrite/diagnóstico , Adolescente , Antirreumáticos/uso terapêutico , Artrite Juvenil/tratamento farmacológico , Criança , Feminino , Seguimentos , França , Humanos , Masculino , Prognóstico , Estudos Retrospectivos , Reumatologia , Fatores de Risco , Sacroileíte/epidemiologia , Sacroileíte/etiologia , Espondilartrite/complicações , Espondilartrite/tratamento farmacológico , Análise de Sobrevida , Centros de Atenção Terciária
20.
Arch Dis Child ; 102(4): 316-322, 2017 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-27655660

RESUMO

AIM: Childhood arthritis arises from several causes. The aim of this observational study is to compare the clinical and biological features and short-term outcome of different types of arthritis because they have different treatment and prognoses. METHODS: Children <16 years of age hospitalised in a French tertiary care centre for a first episode of arthritis lasting for less than 6 weeks who underwent joint aspiration were retrospectively included. We performed non-parametrical tests to compare groups (septic arthritis (SA), juvenile idiopathic arthritis (JIA) and arthritis with no definitive diagnosis). The time before apyrexia or C reactive protein (CRP) <10 mg/L was analysed using the Kaplan-Meier method. RESULTS: We studied 125 children with a sex ratio (M/F) of 1.1 and a median age of 2.2 years (range 0.3 to 14.6). SA was associated with a lower age at onset (1.5 years, IQR 1.2-3.0 vs 3.6 years, IQR 2.2-5.6), shorter duration of symptoms before diagnosis (2 days, IQR 1-4 vs 7 days, IQR 1-19) and higher synovial white blood cell count (147 cells ×103/mm3, IQR 71-227, vs 51 cells ×103/mm3, IQR 12-113), than JIA. Apyrexia occurred later in children with JIA (40% after 2 days, 95% CI 17% to 75%) than children with SA (82%, 95% CI 68% to 92%), as did CRP<10 mg/L (18% at 7 days, 95% CI 6.3% to 29.6% vs 82.1%, 95% CI 76.1% to 89.7%, p=0.01). CONCLUSIONS: There were no sufficiently reliable predictors for differentiating between SA and JIA at onset. The outcomes were different; JIA should be considered in cases of poor disease evolution after antibiotic treatment and joint aspiration.


Assuntos
Artrite Infecciosa/diagnóstico , Artrite Juvenil/diagnóstico , Adolescente , Idade de Início , Antibacterianos/uso terapêutico , Artrite Infecciosa/tratamento farmacológico , Artrite Infecciosa/microbiologia , Artrite Juvenil/tratamento farmacológico , Artrite Juvenil/microbiologia , Biópsia por Agulha , Proteína C-Reativa/metabolismo , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Hospitalização , Humanos , Lactente , Masculino , Estudos Retrospectivos , Líquido Sinovial/química
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