Skip to main content

Main menu

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • ERS Guidelines
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • Peer reviewer login
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • ERS Guidelines
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • Peer reviewer login
  • Alerts
  • Subscriptions

Clinical features and prognosis of 108 patients with lymphangioleiomyomatosis

Yoshikazu Inoue, Masaki Hirose, Koh Nakata, Toru Arai, Chikatoshi Sugimoto, Yoshinobu Matsuda, Naoko Takeuchi, Aya Hirooka, Kazunobu Tachibana, Akiko Matsumuro, Tomomi Honnma, Masanori Kitaichi, Masanori Akira, Masaji Okada, Seiji Hayashi
European Respiratory Journal 2014 44: 3221; DOI:
Yoshikazu Inoue
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Masaki Hirose
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Koh Nakata
2Bioscience Medical Research Center, Niigata University Medical and Dental Hospital, Niigata, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Toru Arai
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Chikatoshi Sugimoto
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Yoshinobu Matsuda
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Naoko Takeuchi
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Aya Hirooka
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Kazunobu Tachibana
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Akiko Matsumuro
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Tomomi Honnma
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Masanori Kitaichi
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Masanori Akira
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Masaji Okada
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
Seiji Hayashi
1Clinical Research Center, and Internal Medicine, National Hospital Organization Kinki-Chuo Chest Medical Center, Sakai, Osaka, Japan
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Info & Metrics
Loading

Abstract

[Introduction]

Lymphangioleiomyomatosis (LAM) is a rare multisystem disease predominantly affecting premenopausal women. Previous reports showed that age, lung function, hormone therapy, pneumothorax, or pulmonary cystic changes was associated with prognosis. To clarify prognostic factors, we analyzed the clinical features and prognostic from our cohort.

[Subjects and Methods]

108 patients {Japanese female, sporadic LAM (n=93) and TSC-LAM (n=15), female} were consecutively registered in NHO Kinki-Chuo Chest Medical Center Cohort until 2013. The age (onset) was 36(30-45) {median(IQR)} years old. We retrospectively analyzed the clinical features and prognosis.

[Results and conclusion]

Angiomyolipomas and lymphangioleiomyomas were detected in 31 and 28 patients. 48 patients had pneumothoraces and 5 patients had chylous effusion. 27 patients were treated with Gn-RH analogues and 39 patients with sirolimus. Initial %FEV1.0 was 78(57-104) %, %DLco was 60(39-77) %. Serum VEGF-D level was 1786(781-3610) pg/ml. Eight patients died from respiratory failure (n=6), abdominal bleeding (n=1), and seizure (n=1), and 5 patients had lung transplantation. Estimated 10-year survival free from death and transplant from the registration (survival) was 73%. There was no significant difference of survival between sporadic LAM and TSC-LAM. Serum VEGF-D < 1000 pg/ml, %FEV1.0 ≥ 70%, and %DLco ≥ 60%, were associated with better prognosis (p<0.05, Log-rank). Menopause and hormone therapy were associated with poor survival.

The prognosis was relatively better than previous reports. Development of new drugs such as mTOR inhibitors are expected to make more improvement.

  • Orphan disease
  • Interstitial lung disease
  • Adolescents
  • © 2014 ERS
Previous
Back to top
Vol 44 Issue Suppl 58 Table of Contents
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Clinical features and prognosis of 108 patients with lymphangioleiomyomatosis
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Citation Tools
Clinical features and prognosis of 108 patients with lymphangioleiomyomatosis
Yoshikazu Inoue, Masaki Hirose, Koh Nakata, Toru Arai, Chikatoshi Sugimoto, Yoshinobu Matsuda, Naoko Takeuchi, Aya Hirooka, Kazunobu Tachibana, Akiko Matsumuro, Tomomi Honnma, Masanori Kitaichi, Masanori Akira, Masaji Okada, Seiji Hayashi
European Respiratory Journal Sep 2014, 44 (Suppl 58) 3221;

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
Clinical features and prognosis of 108 patients with lymphangioleiomyomatosis
Yoshikazu Inoue, Masaki Hirose, Koh Nakata, Toru Arai, Chikatoshi Sugimoto, Yoshinobu Matsuda, Naoko Takeuchi, Aya Hirooka, Kazunobu Tachibana, Akiko Matsumuro, Tomomi Honnma, Masanori Kitaichi, Masanori Akira, Masaji Okada, Seiji Hayashi
European Respiratory Journal Sep 2014, 44 (Suppl 58) 3221;
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo

Jump To

  • Article
  • Info & Metrics
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • Acute hyperoxic challenge improves haemodynamics & Pulmonary vascular stiffness in interstitial lung disease-associated pulmonary hypertension
  • Usual interstitial pneumonia preceding rheumatoid arthritis: Clinical, imaging, and histopathologic features
  • Serum surfactant protein D is a potential biomarker of lung damage in systemic sclerosis
Show more 1.5 Diffuse Parenchymal Lung Disease

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About the ERJ

  • Journal information
  • Editorial board
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • Podcasts
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Print ISSN:  0903-1936
Online ISSN: 1399-3003

Copyright © 2023 by the European Respiratory Society