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

• DOI: 10.1164/rccm.201709-1965st
• Publication Date: 2017-11-15T15:32:52Z
• Authors: Nishant Gupta; Geraldine A. Finlay; Robert M. Kotloff; Charlie Strange; Kevin C. Wilson; Lisa R. Young; Angelo M. Taveira...; Simon R. Johnson; Vincent Cottin; Steven A. Sahn; Jay H. Ryu; Kuniaki Seyama; Yoshikazu Inoue; Gregory P. Downey; MeiLan K. Han; Thomas V. Colby; Kathryn A. Wikenh...; Cristopher A. Meyer; Karen Smith; Joel Moss; Francis X. McCormack
• Keywords: Guideline; Lymphangioleiomyomatosis; Pleurodesis; Grading (engineering); High-resolution computed tomography

Recommendations regarding key aspects related to the diagnosis and pharmacological treatment of lymphangioleiomyomatosis (LAM) were recently published. We now provide additional recommendations four specific questions LAM management pneumothoraces in patients with LAM.Systematic reviews performed then discussed by a multidisciplinary panel. For each intervention, panel considered its confidence estimated effects, balance desirable (i.e., benefits) undesirable harms burdens) consequences, patient values preferences, cost, feasibility. Evidence-based formulated, written, graded using GRADE (Grading Recommendations, Assessment, Development, Evaluation) approach.For women who have cystic changes on high-resolution computed tomography chest characteristic LAM, but no confirmatory features clinical, radiologic, or serologic), guideline made conditional against making clinical basis findings alone for considering transbronchial lung biopsy as diagnostic tool. The also offering pleurodesis after an initial pneumothorax rather than postponing procedure until first recurrence being used reason exclude from transplantation.Evidence-based are provided. Frequent reassessment updating will be needed.