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Pneumologie 2020; 74(05): 263-293
DOI: 10.1055/a-1120-3531
Leitlinie

S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose

German Guideline for Idiopathic Pulmonary Fibrosis
J. Behr
 1   Medizinische Klinik und Poliklinik V, Klinikum der Universität München und Asklepios Fachkliniken München-Gauting, Comprehensive Pneumology Center, Mitglied des Deutschen Zentrums für Lungenforschung, München
,
A. Günther
 2   Schwerpunkt Fibrosierende Lungenerkrankungen, Universitätsklinikum Gießen – Marburg, Standort Gießen, Justus-Liebig-Universität Gießen, sowie Agaplesion Pneumologische Klinik Waldhof-Elgershausen, Greifenstein, University of Giessen Marburg Lung Center, Mitglied des Deutschen Zentrums für Lungenforschung
,
F. Bonella
 3   Zentrum für interstitielle und seltene Lungenkrankheiten, Klinik für Pneumologie, Ruhrlandklinik, Universitätsmedizin Essen, Essen
,
J. Dinkel
 4   Klinik für Radiologie, Klinikum der Universität München, LMU, und Asklepios Fachkliniken München Gauting, Mitglied des Deutschen Zentrums für Lungenforschung (DZL)
,
L. Fink
 5   Institut für Pathologie und Zytologie, ÜGP Wetzlar, Mitglied des Deutschen Zentrums für Lungenforschung, Wetzlar
,
T. Geiser
 6   Universitätsklinik für Pneumologie, Universitätsspital, Universität Bern, Bern
,
K. Geißler
 7   Patientenvertretung Lungenfibrose e. V., Essen
,
S. Gläser
 8   Vivantes Klinikum Neukölln und Spandau Berlin, Klinik für Innere Medizin – Pneumologie und Infektiologie sowie und Universitätsmedizin Greifswald, Greifswald
,
S. Handzhhiev
 9   Klinische Abteilung für Pneumologie, Universitätsklinikum Krems, Krems an der Donau, Österreich
,
D. Jonigk
10   Institut für Pathologie, Medizinische Hochschule Hannover, Mitglied des Deutschen Zentrums für Lungenforschung, Hannover
,
D. Koschel
11   Abteilung Innere Medizin/Pneumologie, Fachkrankenhaus Coswig, Zentrum für Pneumologie, Allergologie, Beatmungsmedizin, Thoraxchirurgie und Medizinische Klinik 1, Bereich Pneumologie, Universitätsklinikum Carl Gustav Carus, Dresden
,
M. Kreuter
12   Zentrum für interstitielle und seltene Lungenerkrankungen, Pneumologie und Beatmungsmedizin, Thoraxklinik, Universitätsklinikum Heidelberg, Mitglied des Deutschen Zentrums für Lungenforschung, Heidelberg
,
G. Leuschner
 1   Medizinische Klinik und Poliklinik V, Klinikum der Universität München und Asklepios Fachkliniken München-Gauting, Comprehensive Pneumology Center, Mitglied des Deutschen Zentrums für Lungenforschung, München
,
P. Markart
13   Schwerpunkt Fibrosierende Lungenerkrankungen, Universitätsklinikum Gießen – Marburg, Standort Gießen, Justus-Liebig-Universität Gießen, University of Giessen Marburg Lung Center, sowie Campus Fulda Universitätsmedizin Marburg, Med. Klinik V; Mitglied des Deutschen Zentrums für Lungenforschung, Fulda
,
A. Prasse
14   Klinik für Pneumologie, Medizinische Hochschule Hannover und Clinical Research Center Fraunhofer Institut ITEM, Mitglied des Deutschen Zentrums für Lungenforschung, Hannover
,
N. Schönfeld
15   Klinik für Pneumologie, Lungenklinik Heckeshorn, Helios Klinikum Emil von Behring, Berlin
,
J. C. Schupp
16   Pulmonary, Critical Care and Sleep Medicine, Yale School of Medicine, Yale University, New Haven, Connecticut, United States
,
H. Sitter
17   Arbeitsgemeinschaft der Wissenschaftlichen Medizinischen Fachgesellschaften (AWMF), Berlin
,
J. Müller-Quernheim
18   Klinik für Pneumologie, Department Innere Medizin, Medizinische Fakultät, Albert Ludwigs Universität, Freiburg
,
U. Costabel
 3   Zentrum für interstitielle und seltene Lungenkrankheiten, Klinik für Pneumologie, Ruhrlandklinik, Universitätsmedizin Essen, Essen
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S2K-Leitlinie zur Diagnostik der idiopathischen LungenfibrosePneumologie 2020; 74(05): e1-e2
DOI: 10.1055/a-1179-2905

Zusammenfassung

Die idiopathische Lungenfibrose (IPF) ist eine schwerwiegende, chronische Lungenerkrankung, deren Diagnosestellung eine profunde fachliche Expertise verlangt. Seit Veröffentlichung der internationalen IPF-Leitlinie im Jahr 2011 mit Update 2018 haben zahlreiche Studien und technische Fortschritte eine Neubewertung des diagnostischen Vorgehens notwendig gemacht. Angesichts der Indikation, symptomatischen Patienten eine antifibrotische Therapie sofort nach Diagnosestellung zu empfehlen, hat die aktuelle IPF-Diagnostik-Leitlinie zum Ziel, die Diagnose der IPF möglichst ohne Verzögerung, wenig invasiv und zuverlässig zu ermöglichen. Sie beschreibt den typischen klinischen Kontext der IPF sowie die Untersuchungen, die zum Ausschluss bekannter Ursachen einer Lungenfibrose erforderlich sind, einschließlich standardisierter Anamneseerhebung, serologischer Testungen und zellulärer Analyse der bronchoalveolären Lavage. Zentrale Bedeutung kommt dem hochauflösenden Computertomogramm in Dünnschichttechnik zu. Sofern eine Histologie zur Diagnoseeingrenzung erforderlich ist, soll diese in erster Linie mittels der transbronchialen Lungen-Kryobiopsie gewonnen werden. Die chirurgische Lungenbiopsie soll Patienten vorbehalten bleiben, die in ausreichender Verfassung für diesen Eingriff sind und bei denen die endoskopische Diagnostik keine Klärung der Diagnose ergab. Goldstandard ist auch weiterhin die interdisziplinäre Diskussion der erhobenen Befunde, um nach Ausschluss anderer Formen chronisch progredienter Lungenfibrosen die Diagnose der IPF zu stellen.

Abstract

Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease. Diagnosis of IPF requires considerable expertise and experience. Since publication of the international IPF guideline in the year 2011 and Update 2018 several studies and technical advances occurred, which made a new assessment of the diagnostic process mandatory. In view of the antifibrotic drugs which have been approved for the treatment of IPF patients, the goal of this guideline is to foster early, confident and effective diagnosis of IPF. The guideline focusses on the typical clinical setting of an IPF patient and provides tools to exclude known causes of interstitial lung disease including standardised questionnaires, serologic testing and cellular analysis of bronchoalveolar lavage. High resolution computed tomography remains crucial in the diagnostic work-up. If it is necessary to obtain specimen for histology transbronchial lung cryobiopsy is the primary approach, while surgical lung biopsy is reserved for patients who are fit for it and in whom bronchoscopic diagnosis did not provide the information needed. Despite considerable progress, IPF remains a diagnosis of exclusion and multidisciplinary discussion remains the golden standard of diagnosis.



Publication History

Article published online:
30 March 2020

© Georg Thieme Verlag KG
Stuttgart · New York

 

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