RESUMO
Chronic nonbacterial osteomyelitis (CNO) can cause significant morbidity, including bone pain and damage. In the absence of clinical trials, treatments include non-steroidal anti-inflammatory drugs, corticosteroids, TNF-inhibitors (TNFi) and/or bisphosphonates. In a retrospective chart review in the United Kingdom and Germany, we investigated response to TNFi and/or pamidronate. Ninety-one patients were included, receiving pamidronate (n = 47), TNFi (n = 22) or both sequentially (n = 22). Patients with fatigue [p = 0.003] and/or arthritis [p = 0.002] were more frequently treated with TNFi than pamidronate. Both therapies were associated with clinical remission at 6 months, and reduction of bone lesions on MRI at 12 months. While not reaching statistical significance, pamidronate resulted in faster resolution of MRI lesions. Fewer flares were observed with TNFi. Failure to respond to pamidronate was associated with female sex [p = 0.027], more lesions on MRI [p = 0.01] and higher CRP levels [p = 0.03]. Randomized clinical trials are needed to confirm observations and generate evidence.
Assuntos
Difosfonatos , Osteomielite , Difosfonatos/uso terapêutico , Feminino , Humanos , Osteomielite/tratamento farmacológico , Osteomielite/patologia , Pamidronato/uso terapêutico , Estudos Retrospectivos , Resultado do Tratamento , Inibidores do Fator de Necrose TumoralRESUMO
BACKGROUND: The scheduled update to the German S3 guidelines on fibromyalgia syndrome (FMS) by the Association of the Scientific Medical Societies ("Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften", AWMF; registration number 041/004) was planned starting in March 2011. MATERIALS AND METHODS: The development of the guidelines was coordinated by the German Interdisciplinary Association for Pain Therapy ("Deutsche Interdisziplinären Vereinigung für Schmerztherapie", DIVS), 9 scientific medical societies and 2 patient self-help organizations. Eight working groups with a total of 50 members were evenly balanced in terms of gender, medical field, potential conflicts of interest and hierarchical position in the medical and scientific fields. Literature searches were performed using the Medline, PsycInfo, Scopus and Cochrane Library databases (until December 2010). The grading of the strength of the evidence followed the scheme of the Oxford Centre for Evidence-Based Medicine. The formulation and grading of recommendations was accomplished using a multi-step, formal consensus process. The guidelines were reviewed by the boards of the participating scientific medical societies. RESULTS AND CONCLUSION: The diagnosis FMS in children and adolescents is not established. In so-called juvenile FMS (JFMS) multidimensional diagnostics with validated measures should be performed. Multimodal therapy is warranted. In the case of severe pain-related disability, therapy should be primarily performed on an inpatient basis. The English full-text version of this article is available at SpringerLink (under "Supplemental").
Assuntos
Dor Crônica/diagnóstico , Dor Crônica/reabilitação , Fibromialgia/diagnóstico , Fibromialgia/reabilitação , Atividades Cotidianas/classificação , Atividades Cotidianas/psicologia , Adolescente , Transtornos de Ansiedade/diagnóstico , Transtornos de Ansiedade/psicologia , Transtornos de Ansiedade/reabilitação , Criança , Dor Crônica/psicologia , Terapia Combinada , Comorbidade , Comportamento Cooperativo , Transtorno Depressivo/diagnóstico , Transtorno Depressivo/psicologia , Transtorno Depressivo/reabilitação , Medicina Baseada em Evidências , Fibromialgia/psicologia , Alemanha , Humanos , Comunicação Interdisciplinar , Admissão do Paciente , Equipe de Assistência ao Paciente , Qualidade de Vida/psicologia , Centros de ReabilitaçãoRESUMO
TNF inhibitors and other biologicals have greatly expanded the therapeutic options for juvenile idiopathic arthritis (JIA). While the efficacy of etanercept and adalimumab has been proven in randomized controlled clinical trials, their long-term safety remains the subject of ongoing investigations. Reports of leukaemia and tumours in children and adolescents treated with etanercept, infliximab and adalimumab have raised questions about an increased risk for malignancies, with lymphoma accounting for the largest group at 50% of all 48 malignancies reported by the FDA.Consequently, TNF inhibitors should be indicated under careful consideration of individual risk factors, such as increased family occurrence of malignancies, or pre-treatment with carcinogenic substances such as cyclophosphamide. This is particularly true for non-approved substances, and non-approved indications, and for combination therapy of TNF inhibitors with immunosuppressive drugs. On the other hand, however, treatment should not be stopped or started in any patient in whom treatment is necessary due to the current knowledge. Adequate patient information, surveillance and documentation of treatment in the registry of the GKJR is strongly recommended.
Assuntos
Sistemas de Notificação de Reações Adversas a Medicamentos , Artrite Juvenil/tratamento farmacológico , Produtos Biológicos/efeitos adversos , Neoplasias/induzido quimicamente , Sociedades Médicas , Fator de Necrose Tumoral alfa/antagonistas & inibidores , United States Food and Drug Administration , Adolescente , Adulto , Produtos Biológicos/uso terapêutico , Criança , Humanos , Leucemia/induzido quimicamente , Linfoma/induzido quimicamente , Uso Off-Label , Resultado do Tratamento , Estados Unidos , Adulto JovemAssuntos
Antineoplásicos/administração & dosagem , Antirreumáticos/administração & dosagem , Artrite Reumatoide/complicações , Artrite Reumatoide/tratamento farmacológico , Neoplasias/complicações , Neoplasias/tratamento farmacológico , Adolescente , Criança , Feminino , Humanos , Masculino , Reumatologia , Sociedades Médicas , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Estados Unidos , United States Food and Drug AdministrationRESUMO
OBJECTIVES: The objectives of this study were to analyse the literature on Sweet's syndrome in childhood focussing on associated diseases and to suggest possible screening procedures for this group of patients. Furthermore, two new patients with Sweet's syndrome are reported. METHODS: A literature search was performed on Pub med using search terms "sweet* syndrome*" and neutrophil* dermatos*. Patients were subdivided into the following groups: classic/idiopathic, paraneoplastic, and parainflammatory Sweet's syndrome. RESULTS: The literature search revealed 64 patients (including our two patients) who were diagnosed with Sweet's syndrome in childhood and adolescence; 27 (42%) patients were categorized as "classic/idiopathic Sweet's syndrome". In 37 patients (58%) chronic associated diseases were reported. Out of these, 21 (33%) patients were categorized as "parainflammatory Sweet's syndrome" including chronic recurrent multifocal osteomyelitis, vasculitis with aortitis, recurrent infections due to immunodeficiencies, arthritis, and systemic lupus erythematosus. Sixteen (25%) patients were categorized as "paraneoplastic Sweet's syndrome" comprising both malignant and premalignant diseases like leukemia, aplastic anaemia, and Fanconi anaemia. As all five (8%) patients treated with drugs (granulocyte-colony stimulating factor, retinoid acid) suffered from malignant, premalignant, or parainflammatory diseases, these patients were categorized according to the underlying disease. Two new children with Sweet's syndrome and associated diseases are presented here, one of them suffering from recurrent infections and trisomy 21, while the other was diagnosed with CNS vasculitis 5(1/2) years after the primary diagnosis. CONCLUSIONS: Sweet's syndrome should be considered in differential diagnosis of prolonged fever with cutaneous involvement. As most cases of pediatric Sweet's syndrome are associated with other diseases we suggest careful screening and monitoring of these patients especially concerning malignant/premalignant diseases, immunodeficiencies, cardiovascular involvement, autoimmune diseases, and drug associations.
Assuntos
Febre/diagnóstico , Febre/metabolismo , Programas de Rastreamento/métodos , Neutrófilos/metabolismo , Dermatopatias/diagnóstico , Dermatopatias/metabolismo , Síndrome de Sweet/epidemiologia , Síndrome de Sweet/fisiopatologia , Doença Aguda , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Imunoglobulinas/sangue , Lactente , Masculino , Neutropenia/diagnóstico , Neutrófilos/patologia , Índice de Gravidade de Doença , Dermatopatias/patologia , Síndrome de Sweet/diagnósticoRESUMO
Henoch-Schoenlein Purpura (PSH) is the most frequent vasculitis in childhood. Skin, bowel and joints are characteristically involved. Although renal manifestations are common PSH-nephritis is infrequent but characterized by a poor prognosis. In autumn 2005 diagnosis and treatment were discussed at the 8th consensus meeting in Wörlitz. These results are summarized.
Assuntos
Vasculite por IgA , Adolescente , Fatores Etários , Biópsia , Criança , Conferências de Consenso como Assunto , Diagnóstico Diferencial , Seguimentos , Alemanha , Hospitalização , Humanos , Vasculite por IgA/complicações , Vasculite por IgA/diagnóstico , Vasculite por IgA/diagnóstico por imagem , Vasculite por IgA/tratamento farmacológico , Vasculite por IgA/patologia , Vasculite por IgA/terapia , Lactente , Recém-Nascido , Rim/patologia , Nefrite/diagnóstico , Nefrite/etiologia , Prognóstico , Radiografia , Pele/patologia , Fatores de Tempo , UltrassonografiaRESUMO
OBJECTIVE: To describe a registry set up to monitor children treated with etanercept in Germany and Austria. METHODS: Giannini's criteria, duration of morning stiffness, number of swollen, tender and contracted joints, adverse events, and reasons for discontinuation were assessed. RESULTS: 322 patients with juvenile idiopathic arthritis (JIA) and 12 additional patients with non-JIA rheumatic diagnoses were included. Therapeutic efficacy was observed from one month after treatment was started. The number of patients with significant improvement and the degree of improvement increased during the first year. The mean (SD) number of tender and swollen joints decreased from 9 (9) and 8.4 (9) to 3.0 (6.5) and 4.5 (7) after one month, and to 2.2 (5.5) and 3.3 (5.5) after three months; morning stiffness decreased from 45 (65) minutes to 12 (30) and 7 (19) after one and three months (p<0.001 for all). Using Gianinni's criteria of 30%, 50%, and 70% improvement, a therapeutic response in JIA patients was achieved in, respectively, 66%, 54%, and 30% after one month, 78%, 61%, and 38% after three months, and 83%, 72%, and 52% after six months. Therapeutic efficacy was lower in patients with systemic onset arthritis. Overall tolerability was good: in 592 patient treatment-years there were 69 reports of adverse events in 56 patients, including one CNS demyelination. There were no opportunistic infections or lupus-like reactions. Treatment was discontinued in 53 JIA patients, in 25 because of lack of efficacy. CONCLUSION: Etanercept treatment was safe and led to a significant improvement in most JIA patients resistant to conventional treatment.