Interstitielle Lungenerkrankungen

ZusammenfassungInterstitielle Pneumonien sind seltene Erkrankungen, die eine diagnostische Herausforderung darstellen. Erst durch die gemeinsamen Anstrengungen der ERS (European Respiratory Society) und ATS (American Thoracic Society) ist es gelungen, die in Europa und Nordamerika unterschiedliche Nomenklatur der interstitiellen Lungenerkrankungen (Alveolitis vs. interstitielle Pneumonie) im Erwachsenenalter zu vereinheitlichen und die diagnostischen Kriterien klar und eindeutig zu definieren. Die ATS/ERS-Klassifikation aus dem Jahre 2002 umfasst 7 Entitäten: gewöhnliche („usual“) interstitielle Pneumonie (UIP), nichtspezifische interstitielle Pneumonie (NSIP), desquamative interstitielle Pneumonie (DIP), respiratorische Bronchiolitis mit assoziierter interstitieller („lung disease“) Lungenerkrankung (RB-ILD), kryptogen organisierende Pneumonie (COP), lymphozytäre interstitielle Pneumonie (LIP), akute interstitielle Pneumonie (AIP). Problematisch ist die Anwendung der ATS/ERS-Klassifikation bei interstitiellen Lungenerkrankungen von Frühgeborenen, Säuglingen und Kindern, da eine UIP, RB-ILD und AIP in diesem Lebensabschnitt nicht vorkommen. Obwohl Säuglinge mit einer schweren respiratorischen Insuffizienz ein morphologisch der DIP oder NSIP ähnliches Bild aufweisen können, sollte diese Krankheitsentität aufgrund der Unterschiede in der Ätiologie, Pathogenese und Prognose als chronische Pneumonitis des Säuglings („chronic pneumonitis of infancy“/CPI) klassifiziert werden.AbstractInterstitial pneumonia is a rare disease, posing a diagnostic challenge to pneumologists, pediatricians, radiologists and pathologists. Only by the combined efforts of the European Respiratory Society (ERS) and the American Thoracic Society (ATS) has has been possible to standardize the formerly different European and Northern American nomenclature of interstitial lung diseases (alveolitis versus interstitial pneumonia) in adults and to clearly and unambiguously define the diagnostic criteria. The ATS/ERS classification of 2002 comprises seven entities: usual interstitial pneumonia (UIP), non-specific interstitial pneumonia (NSIP), desquamative interstitial pneumonia (DIP), respiratory bronchiolitis-associated interstitial lung disease (RB-ILD), cryptogenic organizing pneumonia (COP), lymphocyte interstitial pneumonia (LIP), and acute interstitial pneumonia (AIP). Using the ATS/ERS classification of interstitial pulmonary diseases in premature infants, infants and children is problematic, since UIP, RB-ILD and AIP do not occur at this age. Although infants with severe respiratory insufficiency may sometimes show morphological features similar to DIP or NSIP, this entity should rather be classified as chronic pneumonitis of infancy (CPI) because of differences in etiology, pathogenesis and prognostic outcome.

• Publication year

  2006

• Venue

  Der Pathologe

• Publication date

  2006-03-01

• Fields of study

  Not labeled

• Identifiers
  DOI 10.1007/s00292-006-0823-4PMID 16456642PMCID 7101537
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

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