Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline
Lymphangioleiomyomatosis Diagnosis and Management: High-Resolution Chest Computed Tomography, Transbronchial Lung Biopsy, and Pleural Disease Management An Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guideline
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DOI：
10.1164/rccm.201709-1965st
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发表时间：
2017-11-15
影响因子：
24.7
通讯作者：
McCormack, Francis X.
中科院分区：
文献类型：
--
作者：
Gupta, Nishant;Finlay, Geraldine A.;McCormack, Francis X.
工作背景：最近发表了关于淋巴管平滑肌瘤病（LAM）诊断和药物治疗关键方面的建议。我们现在提供额外的建议，关于四个具体的问题有关的诊断LAM和管理的LAM.Methods患者气胸：进行系统评价，然后讨论了多学科小组。对于每一种干预措施，专家组考虑了其对估计效果的信心、理想的平衡（即，益处）和不期望的（即，危害和负担）后果、患者价值观和偏好、成本和可行性。循证建议，然后制定，书面和分级使用等级（分级的建议，评估，发展和评价）approaches.Results：对于妇女谁有囊性变化的高分辨率计算机断层扫描的胸部特征的LAM，但谁没有额外的确证功能的LAM（即，临床、放射学或血清学），指南小组提出了条件性建议，反对仅基于高分辨率计算机断层扫描结果进行LAM的临床诊断，并考虑将经支气管肺活检作为诊断工具。该指南小组还提出了有条件的建议，提供胸膜固定术后，最初的气胸，而不是推迟的程序，直到第一次复发，并对胸膜固定术被用来作为一个理由，以排除患者的肺transplantation.Conclusions：证据为基础的建议与LAM患者的诊断和治疗。需要经常重新评估和更新。
Background: Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations regarding four specific questions related to the diagnosis of LAM and management of pneumothoraces in patients with LAM.Methods: Systematic reviews were performed and then discussed by a multidisciplinary panel. For each intervention, the panel considered its confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences, patient values and preferences, cost, and feasibility. Evidence-based recommendations were then formulated, written, and graded using the GRADE (Grading of Recommendations, Assessment, Development, and Evaluation) approach.Results: For women who have cystic changes on high-resolution computed tomography of the chest characteristic of LAM, but who have no additional confirmatory features of LAM(i.e., clinical, radiologic, or serologic), the guideline panel made conditional recommendations against making a clinical diagnosis of LAM on the basis of the high-resolution computed tomography findings alone and for considering transbronchial lung biopsy as a diagnostic tool. The guideline panel also made conditional recommendations for offering pleurodesis after an initial pneumothorax rather than postponing the procedure until the first recurrence and against pleurodesis being used as a reason to exclude patients from lung transplantation.Conclusions: Evidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.