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1.
Semin Arthritis Rheum ; 50(6): 1314-1325, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33130459

RESUMO

BACKGROUND: A comprehensive review of outcome measures used in randomized controlled trials (RCTs) of ANCA-associated vasculitis (AAV) could advance trial conductance for this disease. METHODS: A systematic literature review of outcome measures (as specified in methods section as primary and/or secondary outcomes) in RCTs of AAV was conducted. Medline, Cochrane CENTRAL, and ClinicalTrials.gov were searched from inception until April 30, 2019 for RCTs enrolling patients with granulomatosis with polyangiitis and/or microscopic polyangiitis. Outcome measures were organized according to domains (e.g. disease activity) and instruments [e.g. Birmingham Vasculitis Activity Score (BVAS)]. RESULTS: Out of 1101 identified records, 68 RCTs were eligible. Disease activity was an outcome domain collected in 67 (98%) of the RCTs. The BVAS was the most widely used instrument for disease assessment but definitions for remissions and relapse varied for the purpose of primary endpoint definitions. Damage, most often assessed by the Vasculitis Damage Index, was an outcome in 30 (44%) of the RCTs. Mortality was specified as an outcome in 26 (38%) studies. The following outcome domains were assessed: patient-reported outcomes (PROs) in 28 (41%), drug exposure/safety in 58 (85%), and biomarkers [acute phase reactants, ANCA levels] in 24 (35%). Timing for outcome assessment differed substantially, with 3, 6, or 12 months being the most frequent time points. CONCLUSION: Outcome measures used in trials in AAV commonly included vasculitis-specific tools for disease assessment, but with heterogeneity in endpoint-definitions and timing of assessments. Other core outcomes in AAV, including PROs, and damage measures, are often omitted in AAV trials.

2.
Artigo em Inglês | MEDLINE | ID: mdl-33202101

RESUMO

OBJECTIVE: There is an unmet need for reliable, validated, and widely-accepted outcome measures for randomized clinical trials in Behçet syndrome (BS). The Outcome Measures in Rheumatology Clinical Trials (OMERACT) BS Working Group, a large, multi-disciplinary group of experts in BS and patients with BS, worked to develop a Core Set of data-driven outcome measures for use in all clinical trials of BS. METHODS: The Core Domain Set was developed through a comprehensive, iterative, multi-stage project which included a systematic review, a focus group meeting and qualitative patient interviews, a survey among experts in BS, a Delphi exercise involving both patients and physician-experts in BS, and utilization of the data, insight, and feedback generated by these processes to develop a final Core Domain Set. RESULTS: All steps were completed and domains were delineated across the organ systems involved in this disease. Since trials in BS often focus on specific manifestations and not the disease in its entirety, the final proposed Core Set includes 5 domains mandatory for study in all trials in BS (disease activity, new organ involvement, quality of life, adverse events, and death) with additional sub-domains mandatory for study of specific organ-systems. The final Core Set was endorsed at the 2018 OMERACT meeting. CONCLUSION: The Core Set of Domains in BS provides the foundation through which the international research community, including clinical investigators, patients, biopharmaceutical industry, and government regulatory bodies can harmonize the study of this complex disease, compare findings across studies, and advance development of effective therapies.

7.
Ann Rheum Dis ; 79(1): 19-30, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31270110

RESUMO

BACKGROUND: Since the publication of the European League Against Rheumatism (EULAR) recommendations for the management of large vessel vasculitis (LVV) in 2009, several relevant randomised clinical trials and cohort analyses have been published, which have the potential to change clinical care and therefore supporting the need to update the original recommendations. METHODS: Using EULAR standardised operating procedures for EULAR-endorsed recommendations, the EULAR task force undertook a systematic literature review and sought opinion from 20 experts from 13 countries. We modified existing recommendations and created new recommendations. RESULTS: Three overarching principles and 10 recommendations were formulated. We recommend that a suspected diagnosis of LVV should be confirmed by imaging or histology. High dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent) should be initiated immediately for induction of remission in active giant cell arteritis (GCA) or Takayasu arteritis (TAK). We recommend adjunctive therapy in selected patients with GCA (refractory or relapsing disease, presence of an increased risk for glucocorticoid-related adverse events or complications) using tocilizumab. Methotrexate may be used as an alternative. Non-biological glucocorticoid-sparing agents should be given in combination with glucocorticoids in all patients with TAK and biological agents may be used in refractory or relapsing patients. We no longer recommend the routine use of antiplatelet or anticoagulant therapy for treatment of LVV unless it is indicated for other reasons. CONCLUSIONS: We have updated the recommendations for the management of LVV to facilitate the translation of current scientific evidence and expert opinion into better management and improved outcome of patients in clinical practice.


Assuntos
Antirreumáticos/uso terapêutico , Arterite de Células Gigantes/tratamento farmacológico , Glucocorticoides/uso terapêutico , Arterite de Takayasu/tratamento farmacológico , Anticorpos Monoclonais Humanizados/uso terapêutico , Aortite/diagnóstico por imagem , Aortite/tratamento farmacológico , Aortite/patologia , Arterite de Células Gigantes/diagnóstico por imagem , Arterite de Células Gigantes/patologia , Humanos , Metotrexato/uso terapêutico , Arterite de Takayasu/diagnóstico por imagem , Arterite de Takayasu/patologia
8.
Rheumatology (Oxford) ; 59(5): 1011-1020, 2020 05 01.
Artigo em Inglês | MEDLINE | ID: mdl-31529073

RESUMO

OBJECTIVES: Temporal artery biopsy (TAB) is a reference test for the diagnosis of GCA but reveals inflammatory changes only in a subset of patients. The lack of knowledge of TAB sensitivity hampers comparisons with non-invasive techniques such as temporal artery ultrasonography. We performed a systematic literature review and meta-analysis to estimate the sensitivity of TAB in GCA and to identify factors that may influence the estimate. METHODS: A systematic literature review involved searching electronic databases and cross-references. Eligibility criteria included publications reporting at least 30 GCA cases fulfilling the original or modified 1990 ACR classification criteria. The pooled proportion of TAB-positive GCA cases was calculated by using aggregated-data meta-analysis with a random-effects model and assessment of heterogeneity with the I2 statistic. Subgroup analyses and meta-regression were used to examine the effect of patient and study characteristics on TAB positivity. RESULTS: Among 3820 publications screened, 32 studies (3092 patients) published during 1993-2017 were analysed. The pooled proportion of TAB-positive GCA cases was 77.3% (95% CI: 71.8, 81.9%), with high between-study heterogeneity (I2 = 90%). The proportion of TAB-positive cases was slightly higher in publications before than in 2012 and after (P = 0.001). CONCLUSION: The estimated sensitivity of 77% provides indirect evidence that TAB is not less sensitive than temporal artery imaging. The unexplained high between-study heterogeneity could result from differences in TAB sampling, processing or interpretation. The decrease in TAB-positive GCA cases over time could reflect an increasing propensity for clinicians to accept a GCA diagnosis without proof by TAB.


Assuntos
Arterite de Células Gigantes/patologia , Artérias Temporais/patologia , Idoso , Biópsia por Agulha , Feminino , Arterite de Células Gigantes/diagnóstico , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Sensibilidade e Especificidade
9.
Rheumatology (Oxford) ; 59(1): 120-128, 2020 01 01.
Artigo em Inglês | MEDLINE | ID: mdl-31382293

RESUMO

OBJECTIVE: Few data are available on the epidemiology and management of GCA in real life. We aimed to address this situation by using health insurance claims data for France. METHODS: This retrospective study used the Echantillon Généraliste de Bénéficiaires (EGB) database, a 1% representative sample of the French national health insurance system. The EGB contains anonymous data on long-term disease status, hospitalizations and reimbursement claims for 752 717 people. Data were collected between 2007 and 2015. The index date was defined as the date of the first occurrence of a GCA code. Demographics, comorbidities, diagnostic tests and therapies were analysed. Annual incidence rates were calculated, and incident and overall GCA cases were studied. RESULTS: We identified 241 patients with GCA. The annual incidence was 7-10/100 000 people ⩾50 years old. Among the 117 patients with incident GCA, 74.4% were females, with mean age 77.6 years and mean follow-up 2.2 years. After the index date, 51.3% underwent temporal artery biopsy and 29.1% high-resolution Doppler ultrasonography. Among the whole cohort, 84.3% used only glucocorticoids. The most-prescribed glucocorticoid-sparing agent was methotrexate (12.0%). CONCLUSION: The incidence of GCA in France is 7-10/100 000 people ⩾ 50 years old. Adjunct agents, mainly methotrexate, are given to only a few patients. The use of temporal artery biopsy in only half of the patients might reflect a shift toward the use of imaging techniques to diagnose GCA.


Assuntos
Antirreumáticos/uso terapêutico , Biópsia/estatística & dados numéricos , Arterite de Células Gigantes/epidemiologia , Metotrexato/uso terapêutico , Ultrassonografia Doppler/estatística & dados numéricos , Idoso , Biópsia/métodos , Bases de Dados Factuais , Feminino , França/epidemiologia , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/tratamento farmacológico , Humanos , Incidência , Masculino , Programas Nacionais de Saúde , Estudos Retrospectivos , Artérias Temporais/patologia
10.
N Engl J Med ; 381(20): 1918-1928, 2019 11 14.
Artigo em Inglês | MEDLINE | ID: mdl-31722152

RESUMO

BACKGROUND: The small-molecule phosphodiesterase 4 inhibitor apremilast modulates cytokines that are up-regulated in Behçet's syndrome. In a phase 2 trial involving patients with Behçet's syndrome, apremilast reduced the incidence and severity of oral ulcers. Data on the efficacy and safety of apremilast in patients with Behçet's syndrome who had active oral ulcers and had not previously received biologic agents are limited. METHODS: In a phase 3 trial, we randomly assigned, in a 1:1 ratio, patients who had Behçet's syndrome with active oral ulcers but no major organ involvement to receive either apremilast at a dose of 30 mg or placebo, administered orally, twice daily for 12 weeks, followed by a 52-week extension phase. The primary end point was the area under the curve (AUC) for the total number of oral ulcers during the 12-week placebo-controlled period (with lower values indicating fewer ulcers). There were 13 secondary end points, including complete response of oral ulcers, change from baseline in pain associated with oral ulcers, disease activity, and change from baseline in the Behçet's Disease Quality of Life score (range, 0 to 30, with higher scores indicating greater impairment in quality of life). Safety was also assessed. RESULTS: A total of 207 patients underwent randomization (104 patients to the apremilast group and 103 to the placebo group). The AUC for the number of oral ulcers was 129.5 for apremilast, as compared with 222.1 for placebo (least-squares mean difference, -92.6; 95% confidence interval [CI], -130.6 to -54.6; P<0.001). The change from baseline in the Behçet's Disease Quality of Life score was -4.3 points in the apremilast group, as compared with -1.2 points in the placebo group (least-squares mean difference, -3.1 points; 95% CI, -4.9 to -1.3). Adverse events with apremilast included diarrhea, nausea, and headache. CONCLUSIONS: In patients with oral ulcers associated with Behçet's syndrome, apremilast resulted in a greater reduction in the number of oral ulcers than placebo but was associated with adverse events, including diarrhea, nausea, and headache. (Funded by Celgene; ClinicalTrials.gov number, NCT02307513.).


Assuntos
Anti-Inflamatórios não Esteroides/uso terapêutico , Síndrome de Behçet/tratamento farmacológico , Úlceras Orais/tratamento farmacológico , Inibidores da Fosfodiesterase 4/uso terapêutico , Talidomida/análogos & derivados , Administração Oral , Adulto , Anti-Inflamatórios não Esteroides/efeitos adversos , Área Sob a Curva , Síndrome de Behçet/complicações , Método Duplo-Cego , Feminino , Humanos , Masculino , Úlceras Orais/etiologia , Inibidores da Fosfodiesterase 4/efeitos adversos , Qualidade de Vida , Talidomida/efeitos adversos , Talidomida/uso terapêutico
11.
RMD Open ; 5(2): e001003, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31673411

RESUMO

Objectives: To analyse the current evidence for the management of large vessel vasculitis (LVV) to inform the 2018 update of the EULAR recommendations. Methods: Two systematic literature reviews (SLRs) dealing with diagnosis/monitoring and treatment strategies for LVV, respectively, were performed. Medline, Embase and Cochrane databases were searched from inception to 31 December 2017. Evidence on imaging was excluded as recently published in dedicated EULAR recommendations. This paper focuses on the data relevant to giant cell arteritis (GCA). Results: We identified 287 eligible articles (122 studies focused on diagnosis/monitoring, 165 on treatment). The implementation of a fast-track approach to diagnosis significantly lowers the risk of permanent visual loss compared with historical cohorts (level of evidence, LoE 2b). Reliable diagnostic or prognostic biomarkers for GCA are still not available (LoE 3b).The SLR confirms the efficacy of prompt initiation of glucocorticoids (GC). There is no high-quality evidence on the most appropriate starting dose, route of administration, tapering and duration of GC (LoE 4). Patients with GCA are at increased risk of dose-dependent GC-related adverse events (LoE 3b). The addition of methotrexate or tocilizumab reduces relapse rates and GC requirements (LoE 1b). There is no consistent evidence that initiating antiplatelet agents at diagnosis would prevent future ischaemic events (LoE 2a). There is little evidence to guide monitoring of patients with GCA. Conclusions: Results from two SLRs identified novel evidence on the management of GCA to guide the 2018 update of the EULAR recommendations on the management of LVV.


Assuntos
Cegueira/prevenção & controle , Arterite de Células Gigantes/complicações , Arterite de Células Gigantes/tratamento farmacológico , Arterite de Takayasu/tratamento farmacológico , Anticorpos Monoclonais Humanizados/administração & dosagem , Anticorpos Monoclonais Humanizados/uso terapêutico , Antirreumáticos/administração & dosagem , Antirreumáticos/uso terapêutico , Biomarcadores/metabolismo , Quimioterapia Combinada , Feminino , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/metabolismo , Glucocorticoides/administração & dosagem , Glucocorticoides/uso terapêutico , Humanos , Masculino , Metotrexato/administração & dosagem , Metotrexato/uso terapêutico , Estudos Observacionais como Assunto , Avaliação de Resultados em Cuidados de Saúde , Ensaios Clínicos Controlados Aleatórios como Assunto , Recidiva , Gestão de Riscos , Vasculite Sistêmica/patologia , Arterite de Takayasu/complicações
12.
RMD Open ; 5(2): e001020, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31673416

RESUMO

Objective: To collect available evidence on management of large vessel vasculitis to inform the 2018 update of the EULAR management recommendations. Methods: Two independent systematic literature reviews were performed, one on diagnosis and monitoring and the other on drugs and surgical treatments. Using a predefined PICO (population, intervention, comparator and outcome) strategy, Medline, Embase and Cochrane databases were accessed. Eligible papers were reviewed and results condensed into a summary of findings table. This paper reports the main results for Takayasu arteritis (TAK). Results: A total of 287 articles were selected. Relevant heterogeneity precluded meta-analysis. Males appear to have more complications than females. The presence of major complications, older age, a progressive disease course and a weaker inflammatory response are associated with a more unfavourable prognosis. Evidence for details on the best disease monitoring scheme was not found. High-quality evidence to guide the treatment of TAK was not found. Glucocorticoids are widely accepted as first-line treatment. Conventional immunosuppressive drugs and tumour necrosis factor inhibitors were beneficial in case series and uncontrolled studies. Tocilizumab failed the primary endpoint (time to relapse) in a randomised controlled clinical trial; however, results still favoured tocilizumab over placebo. Vascular procedures may be required, and outcome is better when performed during inactive disease. Conclusions: Evidence to guide monitoring and treatment of patients with TAK is predominantly derived from observational studies with low level of evidence. Therefore, higher-quality studies are needed in the future.


Assuntos
Arterite de Takayasu/diagnóstico , Arterite de Takayasu/terapia , Biomarcadores , Terapia Combinada , Comorbidade , Diagnóstico Diferencial , Gerenciamento Clínico , Suscetibilidade a Doenças , Arterite de Células Gigantes/diagnóstico , Arterite de Células Gigantes/etiologia , Arterite de Células Gigantes/terapia , Humanos , Assistência Centrada no Paciente , Prognóstico , Índice de Gravidade de Doença , Avaliação de Sintomas , Arterite de Takayasu/etiologia , Resultado do Tratamento
14.
J Rheumatol ; 46(9): 1198-1201, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30877212

RESUMO

OBJECTIVE: The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working Group seeks to develop validated outcome measures for use in trials for large-vessel vasculitis (LVV). METHODS: An international Delphi exercise conducted among investigators identified items considered important to measure active disease. In parallel, qualitative research with patients was conducted, including interviews and focus groups. RESULTS: Next steps prioritized by the group for LVV include (1) defining disease states (remission, flare, and patient-acceptable symptom state) and (2) selection of patient-reported outcome tools. CONCLUSION: The ultimate goal is to develop an OMERACT-endorsed core set of outcome measures for use in clinical trials of LVV.


Assuntos
Arterite de Células Gigantes/diagnóstico , Arterite de Takayasu/diagnóstico , Humanos , Avaliação de Resultados em Cuidados de Saúde , Medidas de Resultados Relatados pelo Paciente , Reumatologia , Índice de Gravidade de Doença
15.
Ann Rheum Dis ; 78(9): 1160-1166, 2019 09.
Artigo em Inglês | MEDLINE | ID: mdl-30898837

RESUMO

Giant cell arteritis (GCA) represents the most common form of primary systemic vasculitis and is frequently associated with comorbidities related to the disease itself or induced by the treatment. Systematically collected data on disease course, treatment and outcomes of GCA remain scarce. The aim of this EULAR Task Force was to identify a core set of items which can easily be collected by experienced clinicians, in order to facilitate collaborative research into the course and outcomes of GCA. A multidisciplinary EULAR task force group of 20 experts including rheumatologists, internists, epidemiologists and patient representatives was assembled. During a 1-day meeting, breakout groups discussed items from a previously compiled collection of parameters describing GCA status and disease course. Feedback from breakout groups was further discussed. Final consensus was achieved by means of several rounds of email discussions after the meeting. A three-round Delphi survey was conducted to determine a core set of parameters including the level of agreement. 117 parameters were regarded as relevant. Potential items were subdivided into the following categories: General, demographics, GCA-related signs and symptoms, other medical conditions and treatment. Possible instruments and assessment intervals were proposed for documentation of each item. To facilitate implementation of the recommendations in clinical care and clinical research, a minimum core set of 50 parameters was agreed. This proposed core set intends to ensure that relevant items from different GCA registries and databases can be compared for the dual purposes of facilitating clinical research and improving clinical care.


Assuntos
Pesquisa Biomédica/normas , Assistência à Saúde/normas , Arterite de Células Gigantes/terapia , Guias de Prática Clínica como Assunto , Reumatologia/normas , Sociedades Médicas , Europa (Continente) , Humanos
16.
Semin Arthritis Rheum ; 48(4): 752-762, 2019 02.
Artigo em Inglês | MEDLINE | ID: mdl-29954598

RESUMO

OBJECTIVES: The aim of this systematic review was to inform the update of European League Against Rheumatism (EULAR) Recommendations for the management of Behçet's syndrome (BS), on the evidence for the treatment of skin, mucosa and joint involvement of BS. METHODS: A systematic literature search, data extraction, statistical analyses and assessment of the quality of evidence were performed according to a pre-specified protocol using the PRISMA guidelines. Studies that assessed the efficacy of an intervention in comparison to an active comparator or placebo for oral ulcers, genital ulcers, papulopustular lesions, nodular lesions or arthritis were included. Where possible, risk ratios were calculated for binary outcomes and mean difference for continuous outcomes. RESULTS: Among the 3927 references that were screened, 37 were included in the analyses. Twenty-seven of these assessed mucocutaneous and 17 assessed joint involvement. Twenty-one of these studies were randomised controlled trials (RCTs). RCTs with colchicine, azathioprine, interferon-alpha, thalidomide, etanercept and apremilast showed beneficial results with some differences according to lesion type and gender. These agents were generally well tolerated with few adverse events causing withdrawal from the study. CONCLUSIONS: RCTs comprised more than a half (21/37, 57%) of the sources included in the evidence synthesis related to skin, mucosa and joint involvement applicable for the EULAR Recommendations for the management of BS. Differences in the outcome measures that were used across the included studies often made it difficult to combine and compare the results.


Assuntos
Síndrome de Behçet/tratamento farmacológico , Imunossupressores/uso terapêutico , Síndrome de Behçet/patologia , Humanos , Articulações/patologia , Membrana Mucosa/patologia , Pele/patologia
17.
J Dtsch Dermatol Ges ; 16(12): 1425-1432, 2018 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-30537317

RESUMO

The nomenclature system agreed upon by the Chapel Hill Consensus Conference (CHCC) with respect to the terminology and precise definition of the various types of vasculitis has gained widespread interdisciplinary and international acceptance. As the revised version from 2012 (CHCC 2012) does not address the special features of vasculitis of the skin, it has recently been supplemented with an addendum containing the nomenclature of cutaneous vasculitides (D-CHCC). The present article provides the German translation of the terms and defintions of the D-CHCC as well as additional explanatory comments. The goal is to enable German-speaking health care providers to more readily apply these defined terms - which are based on interdisciplinary consensus - in everyday clinical practice and to facilitate assessment of their practicability and relevance in patient care. If the majority of cutaneous vasculitides diagnosed can be matched with any of the defined entities presented herein, it might subsequently be possible to develop a classification system and diagnostic algorithms.


Assuntos
Dermatopatias Vasculares , Vasculite , Humanos , Dermatopatias Vasculares/diagnóstico , Terminologia como Assunto , Vasculite/diagnóstico
19.
Pediatrics ; 142(5)2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30377240

RESUMO

BACKGROUND AND OBJECTIVES: Immunoglobulin A vasculitis (IgAV) might develop after vaccination. However, this potential relationship is essentially based on case reports, and robust pharmaco-epidemiologic data are scarce. We aimed to investigate the effect of vaccination on short-term risk of IgAV in children. METHODS: We enrolled children <18 years old with IgAV seen in 5 pediatric departments from 2011 to 2016. Data on vaccinations administered during the year preceding IgAV onset were collected from immunization records. With a case-crossover method and by using conditional logistic-regression analyses, odds ratios (ORs) and 95% confidence intervals (CIs) were calculated by comparing vaccine exposure during the 3-month "index period" immediately preceding IgAV onset to that during 3 consecutive 3-month "control" periods immediately before the index period. Stratifications by season, year of onset, infection history, age, sex, type, or number of vaccines were performed. Sensitivity analyses used 1-, 1.5-, or 2-month index and control periods. RESULTS: Among 167 children (mean age: 6.7 years) enrolled, 42 (25%) received ≥1 vaccine during the year before IgAV onset. Fifteen (9%) children were vaccinated during the 3-month index period as compared with 4% to 7% during the 3 control periods. The OR for IgAV occurring within the 3 months after vaccination was 1.6 (95% CI: 0.8-3.0). Analyses of IgAV risk within 1, 1.5, or 2 months of vaccination yielded ORs of 1.4 (95% CI: 0.5-3.5), 1.4 (95% CI: 0.6-3.2), and 1.3 (95% CI: 0.6-2.6), respectively. Stratifications revealed no significant association. CONCLUSIONS: Vaccination may not be a major etiological factor of childhood IgAV.


Assuntos
Púrpura de Schoenlein-Henoch/etiologia , Vacinação/efeitos adversos , Vacinas/efeitos adversos , Adolescente , Estudos de Casos e Controles , Criança , Pré-Escolar , Estudos Cross-Over , Feminino , França , Humanos , Masculino , Medição de Risco/métodos
20.
Rheumatology (Oxford) ; 57(12): 2200-2212, 2018 12 01.
Artigo em Inglês | MEDLINE | ID: mdl-30107448

RESUMO

Objective: To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS. Methods: A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included. Results: We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A. Conclusion: The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.


Assuntos
Síndrome de Behçet/tratamento farmacológico , Oftalmopatias/tratamento farmacológico , Gastroenteropatias/tratamento farmacológico , Imunossupressores/uso terapêutico , Doenças do Sistema Nervoso/tratamento farmacológico , Doenças Vasculares/tratamento farmacológico , Anticoagulantes/uso terapêutico , Síndrome de Behçet/complicações , Ensaios Clínicos como Assunto , Oftalmopatias/etiologia , Gastroenteropatias/etiologia , Glucocorticoides/uso terapêutico , Humanos , Doenças do Sistema Nervoso/etiologia , Guias de Prática Clínica como Assunto , Doenças Vasculares/etiologia
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