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Cited by 590Tuberous Sclerosis Complex ResearchMedical Imaging and Pathology StudiesVascular Malformations and Hemangiomas

The DOI matches its Crossref record; 115 of the 115 references it cites were found in a registry or an index.

European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

Open access
Johnson, S. R., Cordier, J. F., Lazor, R., Cottin, V., Costabel, U., Harari, S., Reynaud-Gaubert, M., Boehler, A., Brauner, M., Popper, H., Bonetti, F., Kingswood, C., the Review Panel of the ERS LAM Task Force·2010·European Respiratory Journal
DOI 10.1183/09031936.00076209
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Abstract

We have generated the first international clinical guidelines for LAM. As LAM is a multisystem rare disease, this required the participation of a range of specialists from many countries, particularly Europe and the USA. The absence of a strong evidence base for this rare disease required the use of a consensus technique to provide guidelines containing the best synthesis of evidence and expert opinion. These guidelines contain the first proposal of diagnostic criteria in LAM and recommend investigations and work-up for patients, screening of groups at risk for LAM, discussion of prognosis and management. The overall aim of this document is to help patients by achieving an earlier diagnosis and better care, including in countries with differing levels of healthcare resources. The guidelines will be reviewed regularly or in real time should a major advance occur.

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Crossref · 10.1183/09031936.00076209

Cites

115 of 115 references verified0 citation errors · 0 not checkable

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  • Fewer patients with lymphangioleiomyomatosis have required lung transplant since standardized use of mTOR inhibitors.

    “Fewer patients with LAM have required lung transplant since the standardized use of mTOR inhibitors as treatment, but it remains an indication for those with severely abnormal lung function (FEV1 < 30%), exertional dyspnea (NYHA class III or IV), or hypoxemia at rest.(303–306, 308–315)”

    From Consensus document for the selection of lung transplant candidates: An update from the International Society for Heart and Lung Transplantation. (2021) · cites this paper as [310]

    The Conclusions recommend against routine sirolimus use for pulmonary LAM outside clinical trials and describe mTOR inhibitors as a possible future treatment, contrary to the claim’s premise of standardized use. They do not report a decline in the number of patients requiring lung transplantation. The transplantation recommendations do support considering transplant for patients with severe disease, but not the claimed decline. (LLM, conf=0.85)

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Citations

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Research works printed but not linked: 21

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  14. 1995, Rev Pneumol Clin, 51, 284
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  17. 2006, Am J Kidney Dis, 47, 95
  18. 2002, Nippon Hinyokika Gakkai Zasshi, 93, 702
  19. 1982, Pathol Annu, 17, 339
  20. Trulock EP. Lung transplantation: special considerations and outcome in LAM. In: Moss J, ed. LAM and other Diseases Characterised by Smooth Muscle Proliferation. New York, Marcel Dekker, 1999; pp. 65–78
  21. 1994, J Heart Lung Transplant, 13, 714

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Metadata

Authors
Johnson, S. R., Cordier, J. F., Lazor, R., Cottin, V., Costabel, U., Harari, S., Reynaud-Gaubert, M., Boehler, A., Brauner, M., Popper, H., Bonetti, F., Kingswood, C., & the Review Panel of the ERS LAM Task Force
Affiliations
  • University of Nottingham (GB)
  • Lyon 1 Université (FR)
  • University of Verona (IT)
ORCID
Venue
European Respiratory Journal
Publisher
European Respiratory Society (ERS)
Published
2009-12-31
Volume
35
Issue
1
Pages
14-26
ISSN
  • 0903-1936 (ISSN-L)
  • 1399-3003
DOI
10.1183/09031936.00076209
OpenAlex
W2099927259
Semantic Scholar
6339b11b8ddd21872703d559033c36b7535105a3
Cited by
590
References
115
Citations by year
2026: 17 · 2025: 46 · 2024: 38 · 2023: 39 · 2022: 30 · 2021: 50 · 2020: 34 · 2019: 39 · 2018: 37 · 2017: 39 · 2016: 36 · 2015: 32 · 2014: 35 · 2013: 29 · 2012: 39
FWCI
19.73
Type
journal article
Language
en
Open access
Locations
  • European Respiratory Journal (publishedVersion)
  • PubMed (publishedVersion)
  • Zurich Open Repository and Archive (University of Zurich) (acceptedVersion)
  • Institutional Research Information System University of Turin (University of Turin) (submittedVersion, other-oa)
  • HAL (submittedVersion)
  • Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
  • IRIS (submittedVersion)
  • SERVAL (Université de Lausanne) (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich) (submittedVersion)
  • Zurich Open Repository and Archive (University of Zurich)
Funders
  • Centre Hospitalier Universitaire Vaudois
  • Karl-Franzens-Universität Graz
  • Université de Lyon
Topics
  • Health Sciences › Medicine › Genetics (clinical) › Tuberous Sclerosis Complex Research
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Medical Imaging and Pathology Studies
  • Health Sciences › Medicine › Surgery › Vascular Malformations and Hemangiomas
Keywords
lymphangioleiomyomatosis, diagnosis, management, pulmonary vascular disease, pulmonary nodules, cystic lung disease
MeSH terms
Humans, Lung Transplantation, Lymphangioleiomyomatosis

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