Abstrakt

The term “pulmonary fibrosis” comprises a vast spectrum of diffuse parenchymal lung diseases (DPLD) with different prognosis and distinct therapeutic options. Some of these diseases have a known (i.e. occupational or environmental) cause but many are etiologically unclear. For several reasons idiopathic pulmonary fibrosis (IPF) represents a unique entity among the DPLD. IPF is one of the most common forms of DPLD, represents the most frequent form of the so-called idiopathic interstitial pneumonias (IIPs), is associated with a very poor prognosis, and is the only form for which the novel antifibrotic compound pirfenidone has been approved. Thus, an accurate diagnosis of IPF and a confident distinction from other DPLD forms is mandatory. Previously, the diagnostic approach to IPF was based on the 2000’ joint statement of the American Thoracic Society (ATS) and European Respiratory Society (ERS) on diagnosis and treatment of IPF. Based on new diagnostic developments and standards and on the results of a substantial number of randomized controlled treatment trials a new evidence-based guideline for diagnosis and management of IPF has recently been established by the ATS/ ERS in collaboration with the Latin American Thoracic Association (ALAT) and the Japanese Respiratory Society (JRS) and has been published in 2011. Very recently, a consensus- based German adaptation of this international guideline became available. The main objectives of this German guideline were to translate, comment and interpret the international guideline in the context of the German Health System. This article gives a brief summary of the state of the art diagnostic algorithm and on therapeutic recommendations for IPF. Similarities and differences between the German and the international guidelines are highlighted.

Autoreninformation

Autoren

• D. von der Beck
• A. Günther
• M. Wygrecka
• P. Markart

Abteilungen

• Medizinische Klinik und Poliklinik II, Universitätsklinikum Gießen und Marburg GmbH, Standort Gießen

Adresse

Prof. Dr. med. P. Markart
Medizinische Klinik und Poliklinik II
Universitätsklinikum Gießen und Marburg GmbH, Standort Gießen
Klinikstraße 33
D-35392 Gießen
Email: [email protected]

Citation