Evidence map›Paper›PMID 30846465›Full record

ArticleThe European respiratory journal2019

Analysis of the MILES cohort reveals determinants of disease progression and treatment response in lymphangioleiomyomatosis.

Nishant Gupta, Hye-Seung Lee, Lisa R Young, Charlie Strange, Joel Moss, Lianne G Singer, Koh Nakata, Alan F Barker, Jeffrey T Chapman, Mark L Brantly and 12 more

Registry-linked trialOpen access · bronzeAbstract readComparative StudyMulticenter Study
In one paragraph

Article in The European respiratory journal, 2019. 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 36 papers, 2 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
36citing papers in PubMed, 2 pooled it
3.9field-weighted citation impact, top 6% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

NCT00414648 phase3completednot on this map

Multicenter International Lymphangioleiomyomatosis Efficacy and Safety of Sirolimus Trial

TypeinterventionalSponsorUniversity of CincinnatiRan2006 to 2011Enrolled89ConditionsLymphangioleiomyomatosisArmsSirolimus, Placebo
3 · Its place in the literature

Who cites it

36 citing papers in PubMed, 2 syntheses or guidelines pooled it, 65 citations in OpenAlex.

  1. Brazilian Thoracic Association recommendations for the management of lymphangioleiomyomatosis.Jornal brasileiro de pneumologia : publicacao oficial da Sociedade Brasileira de Pneumologia e Tisilogia · 2025
    Guideline
  2. Rapamycin and rapalogs for tuberous sclerosis complex.The Cochrane database of systematic reviews · 2023
    Pooled it
  3. Article
  4. Article
  5. Article
  6. Review
  7. LAM-CAM:American journal of respiratory cell and molecular biology · 2025
    Article
  8. Article
  9. Review
  10. Article
  11. Article
  12. Article
  13. Article
  14. Article
  15. Review
  16. Article
  17. A 24-year-Old woman with recurrent pneumothoraces.Respiratory medicine case reports · 2024
    Article
  18. Tuberous sclerosis complex associated lymphangioleiomyomatosis.QJM : monthly journal of the Association of Physicians · 2023
    Article
  19. Lost in translation: a neglected mTOR target for lymphangioleiomyomatosis.European respiratory review : an official journal of the European Respiratory Society · 2023
    Review
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

22 authors at 17 institutions in 3 countries.

Nishant GuptaUniversity of Cincinnati, Cincinnati, OH, USA.
Hye-Seung LeeUniversity of South Florida, Tampa, FL, USA.
Lisa R YoungVanderbilt University Medical Center, Nashville, TN, USA.
Charlie StrangeMedical University of South Carolina, Charleston, SC, USA.
Joel MossNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Lianne G SingerUniversity of Toronto, Toronto, ON, Canada.
Koh NakataNiigata University Medical and Dental Hospital, Niigata, Japan.
Alan F BarkerOregon Health and Science University, Portland, OR, USA.
Jeffrey T ChapmanCleveland Clinic, Abu Dhabi, United Arab Emirates.
Mark L BrantlyUniversity of Florida, Gainesville, FL, USA.
James M StocksUniversity of Texas Health Sciences Center, Tyler, TX, USA.
Kevin K BrownNational Jewish Health and the University of Colorado, Denver, CO, USA.
Joseph P LynchUniversity of California, Los Angeles, CA, USA.
Hilary J GoldbergHarvard Medical School, Boston, MA, USA.
Gregory P DowneyNational Jewish Health and the University of Colorado, Denver, CO, USA.
Angelo M Taveira-DaSilvaNational Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD, USA.
Jeffrey P KrischerUniversity of South Florida, Tampa, FL, USA.
Kenneth SetchellCincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Bruce C TrapnellUniversity of Cincinnati, Cincinnati, OH, USA.
Yoshikazu InoueNational Hospital Organization Kinki-Chuo Chest Medical Center, Osaka, Japan.
Francis X McCormackUniversity of Cincinnati, Cincinnati, OH, USA.
NIH Rare Lung Disease Consortium
Cincinnati Children's Hospital Medical Center · USUniversity of Cincinnati · USUniversity of South Florida · USHarvard University · USMedical University of South Carolina · USNational Heart Lung and Blood Institute · USNational Institutes of Health · USNational Jewish Health · USNational Kinki Chuo Hospital for Chest Disease · JPNiigata University Medical and Dental Hospital · JPOregon Health & Science University · USThe University of Texas Health Science Center at Tyler · USUniversity of California, Los Angeles · USUniversity of Colorado Denver · USUniversity of Florida · USUniversity of Toronto · CAVanderbilt University Medical Center · US

Funding

Oregon Clinical and Translational Research Institute - The National COVID Cohort Collaborative (N3C)UL1TR002369 · NCATS · OREGON HEALTH & SCIENCE UNIVERSITY · PI Cynthia D Morris, Christopher G. Slatore · 2017 to 2026
$78.4M
RARE DISEASES DATA AND TECHNOLOGY COORDINATING CENTERU54RR019259 · NCRR · UNIVERSITY OF SOUTH FLORIDA · PI KRISCHER, JEFFREY P · 2003 to 2008
$14.4M
CTSA INFRASTRUCTURE FOR PEDIATRIC RESEARCHUL1RR026314 · NCRR · UNIVERSITY OF CINCINNATI · PI HEUBI, JAMES E. · 2009 to 2011
$14.3M
Role of GM-CSF in Myeloid Cell Function and Innate ImmunityR01HL085453 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI TRAPNELL, BRUCE C · 2007 to 2023
$6.5M
RARE LUNG DISEASE CLINICAL RESEARCH NETWORKU54RR019498 · NCRR · CINCINNATI CHILDRENS HOSP MED CTR · PI TRAPNELL, BRUCE C · 2003 to 2008
$5.7M
Pulmonary Macrophage Transplantation for Pulmonary Alveolar ProteinosisR01HL118342 · NHLBI · CINCINNATI CHILDRENS HOSP MED CTR · PI TRAPNELL, BRUCE C, TRAPNELL, BRUCE COLSTON · 2014 to 2021
$4.5M
Checkpoint Function of PTPalpha in Pathological Fibrogenesis in the LungR01HL132950 · NHLBI · NATIONAL JEWISH HEALTH · PI DOWNEY, GREGORY PAUL · 2016 to 2019
$2.0M
FDA HHS R01 FD003362NCATS NIH HHS UL1 TR002369NCRR NIH HHS U54 RR019259NCRR NIH HHS U54 RR019498NCRR NIH HHS UL1 RR026314NHLBI NIH HHS R01 HL085453NHLBI NIH HHS R01 HL118342NHLBI NIH HHS R01 HL132950
6 · The paper itself

Abstract

introductionThe Multicenter International Lymphangioleiomyomatosis (LAM) Efficacy of Sirolimus (MILES) trial revealed that sirolimus stabilised lung function in patients with moderately severe LAM. The purpose of this study was to further examine the MILES cohort for the effects of racial, demographic, clinical and physiological patient characteristics on disease progression and treatment response in LAM.

methodsMILES subjects were stratified on the basis of menopausal status (pre-menopausal/post-menopausal), race (Asian/Caucasian), bronchodilator responsiveness (present/absent), initial forced expiratory volume in 1 s (FEV

resultsIn the MILES placebo group, pre-menopausal patients declined 5-fold faster than post-menopausal patients (mean±se FEV

conclusionsIn LAM patients, treatment with sirolimus is beneficial regardless of menopausal status, race, bronchodilator responsiveness, baseline FEV

Indexed as

AdultAntibiotics, AntineoplasticAsian PeopleBronchodilator AgentsCohort StudiesDisease ProgressionFemaleForced Expiratory VolumeHumansLung NeoplasmsLymphangioleiomyomatosisMiddle AgedPostmenopausePremenopauseSirolimusTreatment OutcomeAntibiotics, AntineoplasticBronchodilator AgentsSirolimus

Identifiers

PMID30846465
PMCPMC7394189
OpenAlexW2921455439

What OpenQuestion holds

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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.