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IPAF – „Interstitial pneumonia with autoimmune features“

Unzureichende Evidenz und das Berlin-Brandenburger IPAF-Register

IPAF – interstitial pneumonia with autoimmune features

Insufficient evidence and the Berlin-Brandenburg IPAF register

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Zusammenfassung

Autoimmunerkrankungen mit Lungenbeteiligung sind gut charakterisiert. In der Praxis gibt es diffuse Lungenparenchymerkrankungen unklarer Ursache, aber mit möglichen Hinweisen auf eine Autoimmunerkrankung, ohne dass eine definitive rheumatologische Diagnose gestellt werden kann. In 2015 veröffentlichte eine Arbeitsgruppe der European Respiratory Society (ERS) und der American Thoracic Society (ATS) einen Klassifikationskatalog zur IPAF („interstitial pneumonia with autoimmune features“), nachdem zuvor unterschiedliche Klassifikationssysteme existierten. Diese Arbeit bietet einen Überblick über aktuelle Evidenz zum Thema IPAF sowie einen Ausblick auf das im Aufbau befindliche Berlin-Brandenburger IPAF-Register. Die Evidenz wurde mittels narrativer PubMed-Recherche gesucht. Zielsetzung und Projektdesign des Registers werden dargestellt. Die Evidenz zu IPAF ist limitiert. Nach Veröffentlichung der ERS/ATS-Kriterien wurden nur wenige, retrospektive Evaluationsstudien publiziert. Der Parameterkatalog des Berlin-Brandenburger IPAF-Registers bildet IPAF-Krankheitsverläufe umfassend ab. Eine Online-Datenplattform für prospektive, multizentrische Langzeitbeobachtungen wurde programmiert. Nach einer Pilotphase ist eine Ausweitung des Datenregisters auf die Region Berlin-Brandenburg geplant. Eine exakte Abgrenzung von idiopathischen interstitiellen Pneumonien (IIP), IPAF und Kollagenosen („connective tissue diseases“, CTD) mit pulmonalen Manifestationen ist weiterhin schwierig. Eine noch intensivere multidisziplinäre Zusammenarbeit von Rheumatologen, Pneumologen, Radiologen, Pathologen und Labormedizinern ist daher anzustreben. Zur Verbesserung der Evidenzbasis können prospektive Patientenregister wie das Berlin-Brandenburger IPAF-Register eine wertvolle Hilfe darstellen.

Abstract

Interstitial pneumonia in patients with connective tissue diseases (CTD) is a well-characterized entity. Several patients with interstitial pneumonia, however, show some features of CTD without having a definitive diagnosis of CTD. In 2015, a European Respiratory Society (ERS) and American Thoracic Society (ATS) task force issued a classification catalogue for interstitial pneumonia with autoimmune features (IPAF). Prior to this, divergent classification systems existed. This article provides an overview of current IPAF evidence as well as an outlook on the Berlin-Brandenburg IPAF registry. The evidence was searched for by means of a narrative PubMed search. Objectives and project design of the registry under construction are presented. The evidence for IPAF is limited. After publication of the ERS/ATS criteria, only a few retrospective observational studies were published. The parameter catalogue of the Berlin-Brandenburg IPAF register comprehensively maps the IPAF disease continuum. An online data platform for prospective, multicenter long-term observations has been programmed. After a pilot phase, an extension of the data register to the region of Berlin-Brandenburg is envisaged. A precise differentiation of idiopathic interstitial pneumonia, IPAF and connective tissue diseases with pulmonary manifestations remains difficult. An even more intensive multidisciplinary cooperation of rheumatologists, pulmonologists, radiologists, pathologists and laboratory physicians is therefore desirable. To improve the evidence basis, prospective patient registries such as the Berlin-Brandenburg IPAF registry can be a valuable aid.

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Authors and Affiliations

  1. Klinik für Pneumologie, Lungenklinik Heckeshorn, Helios Klinikum Emil von Behring, Walterhöferstraße 11, 14165, Berlin, Deutschland

    T. Blum, C. Fléchet, N. Schönfeld & T. Bauer

  2. Praxisgemeinschaft Wiegemann & Gerber, Berlin, Deutschland

    A. Gerber

  3. Gewebediagnostik/Pathologie im MVZ am Helios Klinikum Emil von Behring, Berlin, Deutschland

    S. Griff

  4. Institut für Diagnostische und Interventionelle Radiologie, Helios Klinikum Emil von Behring, Berlin, Deutschland

    C. Großwendt

  5. Privatpraxis für Diagnostische Radiologie und Begutachtung, Berlin, Deutschland

    B. Rehbock

  6. Institut für Mikrobiologie, Immunologie und Laboratoriumsmedizin, Helios Klinikum Emil von Behring, Berlin, Deutschland

    H. Rüssmann

  7. Abteilung Rheumatologie und Klinische Immunologie, Immanuel Krankenhaus, Berlin, Deutschland

    A. Krause

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Correspondence to T. Blum.

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Interessenkonflikt

T. Blum, A. Gerber, T. Bauer, C. Fléchet, S. Griff, C. Großwendt, B. Rehbock, H. Rüssmann, N. Schönfeld und A. Krause geben an, dass kein Interessenkonflikt besteht.

Dieser Beitrag beinhaltet keine von den Autoren durchgeführten Studien an Menschen oder Tieren.

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P. Kardos, Frankfurt

W. Randerath, Solingen

T. Blum und A. Gerber teilen sich die Erstautorenschaft. T. Bauer und A. Krause teilen sich die Letztautorenschaft.

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Blum, T., Gerber, A., Fléchet, C. et al. IPAF – „Interstitial pneumonia with autoimmune features“. Pneumologe 15, 103–112 (2018). https://doi.org/10.1007/s10405-017-0164-4

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