GuidelineAmerican journal of respiratory and critical care medicine2016
Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: Lymphangioleiomyomatosis Diagnosis and Management.
Guideline in American journal of respiratory and critical care medicine, 2016. 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 184 papers, 6 of them syntheses that pooled it.
What it found
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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
184 citing papers in PubMed, 6 syntheses or guidelines pooled it.
- 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
- Diagnostic performance of VEGF-D for lymphangioleiomyomatosis: a meta-analysis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2022Pooled it
- A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism.BioMed research international · 2021Pooled it
- The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.Respiratory research · 2020Pooled it
- The efficacy and adverse events of mTOR inhibitors in lymphangioleiomyomatosis: systematic review and meta-analysis.Orphanet journal of rare diseases · 2018Pooled it
- Novel Pulmonary Cyst Characteristics Associated with the Diffusing Capacity of the Lung in Lymphangioleiomyomatosis: A Cross-Sectional Clinical Trial.Annals of the American Thoracic Society · 2023Trial
- Trial
- Article
- A Multicenter Registry for Rare Interstitial Lung Diseases in Korea: Baseline Characteristics and Clinical Outcomes.Tuberculosis and respiratory diseases · 2026Article
- Neural crest cell signatures drive tumorigenesis in tuberous sclerosis complex and lymphangioleiomyomatosis.JCI insight · 2026Article
- Article
- Exploring glucocorticoid receptor signalling in lymphangioleiomyomatosis.ERJ open research · 2026Article
- Three cases of whole breast irradiation for breast cancer in patients with Lymphangioleiomyomatosis (LAM).Breast cancer (Tokyo, Japan) · 2026Article
- Access to lymphangioleiomyomatosis care across Europe: an ERN-LUNG survey of clinical practice, and the case for a European registry and trial network.ERJ open research · 2026Article
- A Case Series of Lymphangioleiomyomatosis, a Rare Cystic Lung Disease.Respirology case reports · 2026Article
- Educational attainment of individuals with lymphangioleiomyomatosis is a determinant of timely diagnosis and treatment uptake.Orphanet journal of rare diseases · 2026Article
- Determinants of adherence to sirolimus among lymphangioleiomyomatosis patients.BMC pulmonary medicine · 2026Article
- Diagnostic Value of Serum VEGF-D in Lymphangioleiomyomatosis: Results of the First Prospective Study in the Russian Federation.Diagnostics (Basel, Switzerland) · 2026Article
- Tuberous sclerosis complex with multisystem involvement: A case report.The Journal of international medical research · 2026Article
124 more citing papers are in PubMed but not listed here.
Corrections and comments
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Authors and funding
33 authors.
Funding
Abstract
backgroundLymphangioleiomyomatosis (LAM) is a rare cystic lung disease that primarily affects women. The purpose of these guidelines is to provide recommendations for the diagnosis and treatment of LAM.
methodsSystematic reviews were performed to summarize evidence pertinent to our questions. The evidence was summarized and discussed by a multidisciplinary panel. Evidence-based recommendations were then formulated, written, and graded using the Grading of Recommendations, Assessment, Development, and Evaluation approach.
resultsAfter considering the panel's confidence in the estimated effects, the balance of desirable (i.e., benefits) and undesirable (i.e., harms and burdens) consequences of treatment, patient values and preferences, cost, and feasibility, recommendations were formulated for or against specific interventions. These included recommendations for sirolimus treatment and vascular endothelial growth factor D testing and recommendations against doxycycline and hormonal therapy.
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.