ArticleAmerican journal of respiratory and critical care medicine2017
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.
Article in American journal of respiratory and critical care medicine, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT00414648 (Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus Trial), which is not on this map. Cited by 128 papers, 3 of them syntheses that pooled it.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus Trial
Who cites it
128 citing papers in PubMed, 3 syntheses or guidelines pooled it, 248 citations in OpenAlex.
- Clinical care in interstitial lung disease: a critical appraisal of clinical guidance documents.European respiratory review : an official journal of the European Respiratory Society · 2025Pooled it
- Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025Guideline
- Probability of sporadic lymphangioleiomyomatosis in women presenting with spontaneous pneumothorax.Orphanet journal of rare diseases · 2023Pooled it
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- Estrogen upregulates NR4A1 to counter TGF beta induced pulmonary fibrosis therapeutic insights for IPF.iScience · 2026Article
- LAMCAK pilot study: urinary cathepsin K as a potential biomarker in lymphangioleiomyomatosis.ERJ open research · 2026Article
- Diagnostic Value of Serum VEGF-D in Lymphangioleiomyomatosis: Results of the First Prospective Study in the Russian Federation.Diagnostics (Basel, Switzerland) · 2026Article
- GPNMB immunohistochemistry is a useful ancillary tool for the diagnosis of pulmonary lymphangioleiomyomatosis.Histopathology · 2026Article
- CCL14, identified by multi-omics approach, serves as a novel indicator of disease severity and progression in lymphangioleiomyomatosis.Orphanet journal of rare diseases · 2026Article
- Non-pharmacological therapies for patients with lymphangioleiomyomatosis: a systematic review and meta-analysis.Frontiers in rehabilitation sciences · 2026Review
- Bilateral Lung Transplantation for Lymphangioleiomyomatosis With Secondary Pulmonary Hemosiderosis: A Case Report.Case reports in pathology · 2026Article
- False positive serum vascular endothelial growth factor-D in a patient with Birt-Hogg-Dubé syndrome.Respiratory medicine case reports · 2026Article
- Lymphangioleiomyomatosis with superimposed organizing pneumonia diagnosed by transbronchial lung cryobiopsy: A case report.Respiratory medicine case reports · 2026Article
- Article
- Diffuse cystic lung diseases-a primer for radiologists.BJR open · 2026Review
- Recurrent Pneumothorax Revealing Lymphangioleiomyomatosis in a Young Woman: A Case Report.Clinical case reports · 2025Article
- Community engagement in health guidelines and other normative products: a methodological review.Bulletin of the World Health Organization · 2025Review
- Article
- Radiological Manifestations of Lymphangioleiomyomatosis: A Local Patient Cohort Analysis.Cureus · 2025Article
68 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
22 authors at 1 institution in 1 country.
Funding
Abstract
backgroundRecommendations 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.
methodsSystematic 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.
resultsFor 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.
conclusionsEvidence-based recommendations for the diagnosis and treatment of patients with LAM are provided. Frequent reassessment and updating will be needed.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.