Idiopathic inflammatory myopathy

特发性炎性肌病
  • 文章类型: Journal Article
    目的:一个国际工作组最近发表了一套关于特发性炎症性肌病(IIM)恶性肿瘤筛查的共识指南。这些指南根据“高”提出了不同的调查策略,“中度”或“标准”恶性肿瘤风险组。这项研究比较了澳大利亚三级转诊中心目前的恶性肿瘤筛查实践与这些指南中概述的建议。
    方法:我们对新诊断的IIM患者进行了回顾性分析。记录有关恶性肿瘤筛查的相关人口统计学和临床数据。使用描述性统计将现有做法与指南进行比较;使用澳大利亚医疗保险福利计划计算费用。
    结果:在确定的47名患者中(66%为女性,中位年龄:63岁[IQR:55.5-70],中位病程:4年[IQR:3-6]),只有1人筛查出恶性肿瘤.20名患者(43%)处于高风险,20人(43%)处于中等风险;其余7人(15%)有IBM,拟议的指南不建议进行筛查。只有3名(6%)患者接受了与国际肌炎评估和临床研究建议完全一致的筛查。大多数(N=39,83%)进行了筛查;其余5名(11%)过度筛查的患者患有IBM。指南不遵守的主要原因是在诊断后的3年内缺乏对高风险个体的重复年度筛查(0%依从性)。筛查的平均费用大大低于遵循指南的预期(每位患者$481.52[SD423.53]vs$1341[SD935.67]),在高危女性患者中观察到的差异最大($2314.29/患者)。
    结论:实施拟议的指南将显著影响临床实践,并导致潜在的额外经济负担。
    OBJECTIVE: An inaugural set of consensus guidelines for malignancy screening in idiopathic inflammatory myopathy (IIM) were recently published by an international working group. These guidelines propose different investigation strategies based on \"high\", \"intermediate\" or \"standard\" malignancy risk groups. This study compares current malignancy screening practices at an Australian tertiary referral center with the recommendations outlined in these guidelines.
    METHODS: We conducted a retrospective analysis of newly diagnosed IIM patients. Relevant demographic and clinical data regarding malignancy screening were recorded. Existing practice was compared with the guidelines using descriptive statistics; costs were calculated using the Australian Medicare Benefit Schedule.
    RESULTS: Of the 47 patients identified (66% female, median age: 63 years [IQR: 55.5-70], median disease duration: 4 years [IQR: 3-6]), only one had a screening-detected malignancy. Twenty patients (43%) were at high risk, while 20 (43%) were at intermediate risk; the remaining seven (15%) had IBM, for which the proposed guidelines do not recommend screening. Only three (6%) patients underwent screening fully compatible with International Myositis Assessment and Clinical Studies recommendations. The majority (N = 39, 83%) were under-screened; the remaining five (11%) overscreened patients had IBM. The main reason for guideline non-compliance was the lack of repeated annual screening in the 3 years post-diagnosis for high-risk individuals (0% compliance). The mean cost of screening was substantially lower than those projected by following the guidelines ($481.52 [SD 423.53] vs $1341 [SD 935.67] per patient), with the highest disparity observed in high-risk female patients ($2314.29/patient).
    CONCLUSIONS: Implementation of the proposed guidelines will significantly impact clinical practice and result in a potentially substantial additional economic burden.
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  • 文章类型: Journal Article
    这项研究的目的是建立基于克罗地亚德尔菲的专家共识,以筛查与结缔组织病(CTD)相关的间质性肺病(ILD)。对ILD发展的危险因素进行了系统的文献综述,ILD的患病率和发病率,ILD的诊断和筛查方法,特发性炎性肌病(IIM)的ILD和预后,混合性结缔组织病(MCTD),原发性干燥综合征(PSS),类风湿性关节炎(RA),系统性红斑狼疮(SLE),和系统性硬化症(SSc)。根据发现的证据,专家开发了用于筛查和监测每个CTD中ILD的问卷,这是通过在线调查提供的。在电子调查之后,基于共识意见,开发了两种筛选算法.ILD的检测策略包括高分辨率计算机断层扫描(HRCT)以及IIM的肺功能测试,MCTD,SSC。和新诊断的pSS的肺功能检测,RA和SLE。然而,在具有ILDHRCT危险因素的患者中,这些测试也应该进行。早期识别各种CTD-ILD患者的筛查策略最初是由风湿病学家的多学科团队开发的,肺病学家,和放射科医生确定早期CTD患者有ILD风险,CTD的严重关节外表现。
    The aim of this study was to develop a Croatian Delphi-based expert consensus for screening interstitial lung disease (ILD) associated with connective tissue disease (CTD). A systematic literature review was conducted on risk factors for the development of ILD, prevalence and incidence of ILD, diagnostic and screening methods for ILD, and prognosis of ILD in idiopathic inflammatory myopathy (IIM), mixed connective tissue disease (MCTD), primary Sjögren\'s syndrome (pSS), rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), and systemic sclerosis (SSc) were performed. Based on the evidence found, experts developed questionnaires for screening and monitoring ILD in each CTD, which were provided via an online survey. Following the electronic survey, two screening algorithms were developed based on the consensus opinions. The detection strategy for ILD included high-resolution computed tomography (HRCT) in addition to pulmonary function testing for IIM, MCTD, and SSc. and pulmonary function testing for newly diagnosed pSS, RA and SLE. However, in patients with identified risk factors for ILD HRCT, these tests should also be performed. A screening strategy for early identification of patients with various CTD-ILD was first developed by a multidisciplinary team of rheumatologists, pulmonologists, and radiologists to identify early CTD patients at risk of ILD, a severe extra-articular manifestation of CTD.
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