Skip to main content
SpringerLink
Log in

Epidemiologie und Register bei seltenen Lungenerkrankungen

  • Chapter
  • First Online:
Seltene Lungenerkrankungen

  • 2098 Accesses

Zusammenfassung

Innerhalb der Europäischen Union werden Erkrankungen als selten definiert (Orphan Diseases, Rare Diseases), wenn ihre Prävalenz < 5 Fällen pro 10.000 Einwohner liegt. Zum Verständnis der Erkrankungshäufigkeit, der Häufigkeitsverteilung sowie der medizinischen Versorgungsrealität sind epidemiologische Studien sowie spezifische Erkrankungsregister von großer Bedeutung. Für viele Entitäten der seltenen Lungenerkrankungen ist das Potenzial dieses Forschungsbereichs jedoch noch nicht ausgeschöpft und bedarf einer größeren Aufmerksamkeit.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 79.99
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Hardcover Book
USD 99.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

Literatur

  • Alpert JS (2000) Are data from clinical registries of any value? Eur Heart J 21(17):1399–1401

    Article  CAS  Google Scholar 

  • Baumgartner KB, Samet JM, Stidley CA, Colby TV, Waldron JA (1997) Cigarette smoking: a risk factor for idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 155(1):242–248

    Article  CAS  Google Scholar 

  • Behr J, Hoeper MM, Kreuter M, Klotsche J, Wirtz H, Pittrow D (2014) Investigating significant health trends in idiopathic pulmonary fibrosis (INSIGHTS-IPF): rationale, aims and design of a nationwide prospective registry. BMJ Open Respir Res 1(1):e000010

    Article  Google Scholar 

  • Behr J et al (2015) Management of patients with idiopathic pulmonary fibrosis in clinical practice: INSIGHTS-IPF. Eur Respir J 46(1):186–196

    Article  Google Scholar 

  • Behr J, Prasse A, Wirtz H, Koschel D, Pittrow D, Held M, Klotsche J, Andreas S, Claussen M, Grohé C, Wilkens H, Hagmeyer L, Skowasch D, Meyer JF, Kirschner J, Gläser S, Kahn N, Welte T, Neurohr C, Schwaiblmair M, Bahmer T, Oqueka T, Frankenberger M, Kreuter M (2020) Survival and course of lung function in the presence or absence of antifibrotic treatment in patients with idiopathic pulmonary fibrosis: long-term results of the INSIGHTS-IPF registry. Eur Respir J 56(2):1902279. https://doi.org/10.1183/13993003.02279-2019

    Article  PubMed  Google Scholar 

  • Cottin V, Annesi-Maesano I, Günther A, Galvin L, Kreuter M, Powell P, Prasse A, Reynolds G, Richeldi L, Spagnolo P, Valenzuela C, Wijsenbeek M, Wuyts WA, Crestani B, Ariane-IPF Clinical Research Collaboration consortium (2019) The Ariane-IPF ERS Clinical Research Collaboration: seeking collaboration through launch of a federation of European registries on idiopathic pulmonary fibrosis. Eur Respir J 53(5):1900539. https://doi.org/10.1183/13993003.00539-2019. Print 2019 May. PMID: 31123022

    Article  PubMed  Google Scholar 

  • Coultas DB, Zumwalt RE, Black WC, Sobonya RE (1994) The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 150(4):967–972

    Article  CAS  Google Scholar 

  • Culver DA, Behr J, Belperio JA, Corte TJ, de Andrade JA, Flaherty KR, Gulati M, Huie TJ, Lancaster LH, Roman J, Ryerson CJ, Kim HJ (2019) Patient registries in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 200(2):160–167

    Article  Google Scholar 

  • Delgado-Rodriguez M, Llorca J (2004) Bias. J Epidemiol Community Health 58(8):635–641

    Google Scholar 

  • Duchemann B, Annesi-Maesano I, Jacobe de Naurois C, Sanyal S, Brillet PY, Brauner M, Kambouchner M, Huynh S, Naccache JM, Borie R et al (2017) Prevalence and incidence of interstitial lung diseases in a multi-ethnic county of Greater Paris. Eur Respir J 50(2):1602419

    Article  Google Scholar 

  • European Society of Cardiology (2015) EURObservational Research Programme. http://www.escardio.org/guidelines-surveys/eorp/Pages/welcome.aspx

  • Ferrara G, Arnheim-Dahlström L, Bartley K, Janson C, Kirchgässler KU, Levine A, Sköld CM (2019) Epidemiology of pulmonary fibrosis: a cohort study using healthcare data in Sweden. Pulm Ther 5(1):55–68. https://doi.org/10.1007/s41030-019-0087-9. Epub 2019 Mar 5. PMID: 32026424

    Article  PubMed  PubMed Central  Google Scholar 

  • Fletcher RH, Fletcher SW, Wagner EH (1996) Clinical epidemiology: the essentials. Lippincott Williams & Wilkins, Philadelphia

    Google Scholar 

  • Gitt AK, Bueno H, Danchin N et al (2010) The role of cardiac registries in evidence-based medicine. Eur Heart J 31(5):525–529

    Article  Google Scholar 

  • Gribbin J, Hubbard RB, Le Jeune I, Smith CJ, West J, Tata LJ (2006) Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax 61(11):980–985

    Article  CAS  Google Scholar 

  • Guenther A, Krauss E, Tello S et al (2018) The European IPF registry (eurIPFreg): baseline characteristics and survival of patients with idiopathic pulmonary fibrosis. Respir Res 19:141

    Article  Google Scholar 

  • Gupta S, Bayoumi AM, Faughnan ME (2011) Rare lung disease research: strategies for improving identification and recruitment of research participants. Chest 140(5):1123–1129

    Article  Google Scholar 

  • Hoeper MM, Huscher D, Ghofrani HA et al (2013) Elderly patients diagnosed with idiopathic pulmonary arterial hypertension: results from the COMPERA registry. Int J Cardiol 168(2):871–880

    Article  Google Scholar 

  • Jo HE, Glaspole I, Grainge C, Goh N, Hopkins PM, Moodley Y, Reynolds PN, Chapman S, Walters EH, Zappala C, Allan H, Keir GJ, Hayen A, Cooper WA, Mahar AM, Ellis S, Macansh S, Corte TJ (2017) Baseline characteristics of idiopathic pulmonary fibrosis: analysis from the Australian Idiopathic Pulmonary Fibrosis Registry. Eur Respir J 49(2):1601592. https://doi.org/10.1183/13993003.01592-2016. Print 2017 Feb. PMID: 28232409

    Article  PubMed  Google Scholar 

  • Korn S, Hübner M, Hamelmann E, Buhl R (2012) Das Register „Schweres Asthma“. Pneumologie 66:341–344

    Article  CAS  Google Scholar 

  • Krauss E, Froehler M, Degen M, Mahavadi P, Dartsch RC, Korfei M, Ruppert C, Seeger W, Guenther A (2019) Exhalative breath markers do not offer for diagnosis of interstitial lung diseases: data from the European IPF Registry (eurIPFreg) and Biobank. J Clin Med 8(5):643. https://doi.org/10.3390/jcm8050643

    Article  CAS  PubMed Central  Google Scholar 

  • Kreuter M, Herth FJF, Wacker M, Leidl R, Hellmann A, Pfeifer M, Behr J, Witt S, Kauschka D, Mall M, Günther A, Markart P (2015) Exploring clinical and epidemiological characteristics of interstitial lung diseases (EXCITING-ILD) – rationale, aims and design of a nationwide prospective registry. Biomed Res Int 2015:123876

    PubMed  PubMed Central  Google Scholar 

  • Luisetti M, Balfour-Lynn IM, Johnson SR et al (2012) Perspectives for improving the evaluation and access of therapies for rare lung diseases in Europe. Respir Med 106(6):759–768

    Article  Google Scholar 

  • Moor CC, Kreuter M, Luppi F, Wuyts WA (2020) The world is not enough – the value of increasing registry data in idiopathic pulmonary fibrosis. Respir Res 21(1):105. https://doi.org/10.1186/s12931-020-01377-1. PMID: 32375778

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • National Center for Advancing Translational Sciences – Office of rare Disease research (2015) [cited]. http://rarediseases.info.nih.gov

  • Olson AL, Gifford AH, Inase N, Fernandez Perez ER, Suda T (2018) The epidemiology of idiopathic pulmonary fibrosis and interstitial lung diseases at risk of a progressive-fibrosing phenotype. Eur Respir Rev 27(150):180077

    Article  Google Scholar 

  • Prevalence of rare diseases (2014a) Bibliographic data. Orphanet report series, rare diseases collection 2014 [cited]. http://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_alphabetical_list.pdf

  • Prevalence of rare diseases (2014b) Bibliographic data. Orphanet report series, rare disease collection 2014 [cited]. http://www.orpha.net/orphacom/cahiers/docs/GB/Prevalence_of_rare_diseases_by_decreasing_prevalence_or_cases.pdf

  • Rademacher J, de Roux A, Ringshausen FC (2015) PROGNOSIS – the PROspective German NOn-CF BronchiectaSIS patient registry. Pneumologie 69(7):391–393. https://doi.org/10.1055/s-0034-1392254

    Article  CAS  PubMed  Google Scholar 

  • Raghu G, Weycker D, Edelsberg J, Bradford WZ, Oster G (2006) Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 174(7):810–816

    Article  Google Scholar 

  • Richter T, Nestler-Parr S, Babela R, Khan ZM, Tesoro T, Molsen E et al (2015) Rare disease terminology and definitions-A systematic global review: report of the ISPOR rare disease special interest group. Value Health 18:906–914

    Article  Google Scholar 

  • Shoemaker-Hunt S, Hall K, Hoffman L (2020) Advancing Patient Safety: Reviews from the Agency for Healthcare Research and Quality’s Making Healthcare Safer III Report. J Patient Saf 16(3 1 Suppl):S1–S2

    Google Scholar 

  • Zurkova M, Kriegova E, Kolek V, Lostakova V, Sterclova M, Bartos V et al (2019) Effect of pirfenidone on lung function decline and survival: 5-yr experience from a real-life IPF cohort from the Czech EMPIRE registry. Respir Res 20(1):16

    Article  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Klinik für Innere Medizin – Pneumologie und Infektiologie, Vivantes Klinikum Neukölln, Berlin, Deutschland

    Sven Gläser

  2. Klinik für Innere Medizin B – Bereich Pneumologie, Infektiologie und Weaningzentrum, Universitätsmedizin Greifswald, Greifswald, Deutschland

    Sven Gläser

  3. Institut für Klinische Pharmakologie, Medizinische Fakultät, Technische Universität Carl Gustav Carus, Dresden, Deutschland

    David Pittrow

  4. Medizinische Klinik und Poliklinik V, Mitglied des Deutschen Zentrums für Lungenforschung (DZL), LMU Klinikum, München, Deutschland

    Jürgen Behr

Authors
  1. Sven Gläser
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. David Pittrow
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jürgen Behr
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Sven Gläser .

Editor information

Editors and Affiliations

  1. Zentrum für interstitielle und seltene Lungenerkrankungen, Thoraxklinik, Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    Michael Kreuter

  2. Ruhrlandklinik, Universitätsmedizin Essen, Essen, Deutschland

    Ulrich Costabel

  3. Thoraxklinik, Pneumologie und Beatmungsmedizin und Translational Lung Research Center (TLRCH), Universitätsklinikum Heidelberg, Heidelberg, Deutschland

    Felix JF Herth

  4. Vormals LungenClinic Grosshansdorf, Grosshansdorf, Deutschland

    Detlef Kirsten

Rights and permissions

Reprints and permissions

Copyright information

© 2022 Springer-Verlag GmbH Deutschland, ein Teil von Springer Nature

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Gläser, S., Pittrow, D., Behr, J. (2022). Epidemiologie und Register bei seltenen Lungenerkrankungen. In: Kreuter, M., Costabel, U., Herth, F.J., Kirsten, D. (eds) Seltene Lungenerkrankungen. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-662-63651-0_1

Download citation

  • DOI: https://doi.org/10.1007/978-3-662-63651-0_1

  • Published:

  • Publisher Name: Springer, Berlin, Heidelberg

  • Print ISBN: 978-3-662-63650-3

  • Online ISBN: 978-3-662-63651-0

  • eBook Packages: Medicine (German Language)

Publish with us

Policies and ethics

Search

Navigation