Charakteristika und Versorgung der idiopathischen Lungenfibrose

 

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Zusammenfassung

Die Idiopathische pulmonale Fibrose (IPF), eine Manifestationsform der chronischen interstitiellen Pneumonie, ist mit einer Prävalenz von ca. 2–29 Fällen pro 100000 Personen eine seltene Erkrankung. Die derzeitigen Behandlungsoptionen sind beschränkt, und die mittlere Überlebenszeit der neu diagnostizierten (meist älteren) Patienten liegt bei nur 2–3 Jahren. Da in europäischen Ländern Daten zur Versorgung der Patienten lückenhaft sind, wurde mit INSIGHTS-IPF in Deutschland ein neues Register initiiert, in dem inzidente und prävalente Patienten mit gesicherter IPF-Diagnose prospektiv dokumentiert werden. Es werden detaillierte Daten zu Patientencharakteristika, diagnostischen Verfahren, Therapie, klinischen Ereignissen, Lebensqualität und Ressourcenverbrauch erfasst. Geplant ist, in ca. 30 Zentren 500 Patienten zu dokumentieren. Das Register wird längerfristig einen Beitrag zur verbesserten Versorgung von Patienten mit IPF leisten.

Abstract

Idiopathic pulmonary fibrosis (IPF), a manifestation of chronic progressive fibrosing interstitial pneumonia, is with a prevalence of 2–29 cases per 100,000 individuals a rare disease. Current treatment options are limited, and the mean survival time of the newly diagnosed (mostly elderly) patients is only about 2–3 years. As in Europe data are limited on the characteristics and management of such patients, INSIGHTS-IPF was initiated as a new registry that documents incident and prevalent patients with confirmed IPF diagnosis prospectively. Detailed data on patient characteristics, diagnostics, management, clinical outcomes, quality of life and resource utilization are recorded. It is planned to document 500 patients in 30 centers. The registry will contribute to the optimization of the management of IPF patients in the long term.

• Literatur

• 1 Brook RH, McGlynn EA, Cleary PD. Measuring quality of care - part two of six. N Engl J Med 1996; 335: 966-970
  CrossrefPubMedGoogle Scholar
• 2 Coultas DB, Zumwalt RE, Black WC et al. The epidemiology of interstitial lung diseases. Am J Respir Crit Care Med 1994; 150: 967-972
  CrossrefPubMedGoogle Scholar
• 3 du Bois RM. Idiopathic pulmonary fibrosis: present understanding and future options. Eur Respir Rev 2011; 20: 132-133
  CrossrefPubMedGoogle Scholar
• 4 Eakin EG, Resnikoff PM, Prewitt LM et al. Validation of a new dyspnea measure: the UCSD Shortness of Breath Questionnaire. University of California, San Diego. Chest 1998; 113: 619-624
  CrossrefPubMedGoogle Scholar
• 5 European Commission. EurIPFnet European IPF Network: Natural course, Pathomechanisms and Novel Treatment Options in Idiopathic Pulmonary Fibrosis. Internet: http://ec.europa.eu/research/health/medical-research/ rare-diseases/projects/euripfnet_en.html Letzter Zugriff 12.03.2012
  PubMedGoogle Scholar
• 6 European Medicines Agency (EMA). Esbriet. CHMP Public Assessment Report EMA/CHMP/115147/2011. London: 16 December 2010. http://www.ema.europa.eu/docs/en_GB/document_library/EPAR_ _Public_assessment_report/human/002154/WC500103073.pdf Letzter Zugriff 22.10.2012
  PubMedGoogle Scholar
• 7 Gemeinsamer Bundesausschuss (G-BA). Zusammenfassende Dokumentation über die Änderung der Arzneimittelrichtlinie (AM-RL): Anlage XII – Beschlüsse über die Nutzenbewertung von Arzneimitteln mit neuen Wirkstoffen nach § 35a SGB V: Pirfenidon. Berlin: 15. März 2012. Internet: http://www.g-ba.de/downloads/40-268-1915/2012-03-15_AM-RL-XII_Pirfenidon_ZD.pdf Letzter Zugriff 22.10.2012
  PubMedGoogle Scholar
• 8 Glatz U. GOLDnet – seltene Lungenerkrankungen im Visier. Dtsch Med Wochenschr 2011; 136: 621
  PubMedGoogle Scholar
• 9 Gribbin J, Hubbard RB, Le Jeune I et al. Incidence and mortality of idiopathic pulmonary fibrosis and sarcoidosis in the UK. Thorax 2006; 61: 980-985
  CrossrefPubMedGoogle Scholar
• 10 Guenther A, Eickelberg O, Preissner KT et al. International registry for idiopathic pulmonary fibrosis. Thorax 2008; 63: 841 author reply 841
  CrossrefPubMedGoogle Scholar
• 11 Iwai K, Mori T, Yamada N et al. Idiopathic pulmonary fibrosis. Epidemiologic approaches to occupational exposure. Am J Respir Crit Care Med 1994; 150: 670-675
  CrossrefPubMedGoogle Scholar
• 12 Kanematsu T, Kitaichi M, Nishimura K et al. Clubbing of the fingers and smooth-muscle proliferation in fibrotic changes in the lung in patients with idiopathic pulmonary fibrosis. Chest 1994; 105: 339-342
  CrossrefPubMedGoogle Scholar
• 13 Karakatsani A, Papakosta D, Rapti A et al. Epidemiology of interstitial lung diseases in Greece. Respir Med 2009; 103: 1122-1129
  CrossrefPubMedGoogle Scholar
• 14 Mura M, Porretta MA, Bargagli E et al. Predicting survival in newly diagnosed idiopathic pulmonary fibrosis. Europ Resp J 2012; 40: 101-109
  PubMedGoogle Scholar
• 15 National Heart Lung and Blood Institute (NHLBI). Commonly used three-drug regimen for idiopathic pulmonary fibrosis found harmful. (NIH News, 21. October 2011). http://www.nih.gov/news/health/ oct2011/nhlbi-21.htm  Letzter Zugriff  22.10.2012
  PubMedGoogle Scholar
• 16 Noble PW, Albera C, Bradford WZ et al. Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY). Lancet 2011; 377: 1760-1769
  CrossrefPubMedGoogle Scholar
• 17 Ohshimo S, Bonella F, Cui A et al. Significance of bronchoalveolar lavage for the diagnosis of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2009; 179: 1043-1047
  CrossrefPubMedGoogle Scholar
• 18 Raghu G. Idiopathic pulmonary fibrosis. A rational clinical approach. Chest 1987; 92: 148-154
  CrossrefPubMedGoogle Scholar
• 19 Raghu G, Collard HR, Egan JJ et al. An official ATS/ERS/JRS/ALAT statement: idiopathic pulmonary fibrosis: evidence-based guidelines for diagnosis and management. Am J Respir Crit Care Med 2011; 183: 788-824
  CrossrefPubMedGoogle Scholar
• 20 Raghu G, Weycker D, Edelsberg J et al. Incidence and prevalence of idiopathic pulmonary fibrosis. Am J Respir Crit Care Med 2006; 174: 810-816
  CrossrefPubMedGoogle Scholar
• 21 Richeldi L, Costabel U, Selman M et al. Efficacy of a tyrosine kinase inhibitor in idiopathic pulmonary fibrosis. N Engl J Med 2011; 365: 1079-1087
  CrossrefPubMedGoogle Scholar
• 22 Scott J, Johnston I, Britton J. What causes cryptogenic fibrosing alveolitis? A case-control study of environmental exposure to dust. BMJ 1990; 301: 1015-1017
  CrossrefPubMedGoogle Scholar
• 23 Selman M, Carrillo G, Estrada A et al. Accelerated variant of idiopathic pulmonary fibrosis: clinical behavior and gene expression pattern. PLoS One 2007; 2: e482
  CrossrefPubMedGoogle Scholar
• 24 Song JW, Hong SB, Lim CM et al. Acute exacerbation of idiopathic pulmonary fibrosis: incidence, risk factors and outcome. Eur Respir J 2011; 37: 356-363
  CrossrefPubMedGoogle Scholar
• 25 Swigris JJ, Brown KK, Behr J et al. The SF-36 and SGRQ: validity and first look at minimum important differences in IPF. Respir Med 2010; 104: 296-304
  CrossrefPubMedGoogle Scholar
• 26 von Plessen C, Grinde O, Gulsvik A. Incidence and prevalence of cryptogenic fibrosing alveolitis in a Norwegian community. Respir Med 2003; 97: 428-435
  CrossrefPubMedGoogle Scholar
• 27 Wilson JW, du Bois RM, King Jr TE. Challenges in pulmonary fibrosis: 8 – The need for an international registry for idiopathic pulmonary fibrosis. Thorax 2008; 63: 285-287
  CrossrefPubMedGoogle Scholar
• 28 World Health Organisation (WHO). http://www.cure4you.dk/354/index.php?m=2&a=e2&ei=4151  Letzter Zugriff 22.10.2012
  PubMed