Related Experiment Video
Updated: Mar 1, 2026
Unilateral Lung Volume Analysis Using Micro-CT for Enhanced Assessment of Pulmonary Fibrosis in Preclinical Models
Published on: June 20, 2025
[German Guideline for Idiopathic Pulmonary Fibrosis - Update on Pharmacological Therapies 2017]
Jürgen Behr1, Andreas Günther2, Francesco Bonella3
1Medizinische Klinik und Poliklinik V, Klinikum der Universität München und Asklepios Fachkliniken München-Gauting, Comprehensive Pneumology Center, Mitglied des Deutschen Zentrums für Lungenforschung.
Abstract:
Idiopathic pulmonary fibrosis (IPF) is a severe and often fatal disease with a median survival of 2 - 4 years after diagnosis. Since the publication of the German IPF guideline in 2013 new treatment trials have been published, necessitating an update of the pharmacological therapy of IPF. Different from the previous guideline, the GRADE system was discarded and replaced by the Oxford evidence classification system which allows a more differentiated judgement. The following pharmacological therapies were rated not suitable for the treatment of IPF patients (recommendation A; evidence 1-b): triple therapy with prednisolone, azathioprine and acetyl-cysteine; imatinib; ambrisentan; bosentan; macitentan. A less clear but still negative recommendation (B, 1-b) was attributed to the treatment of IPF with the phosphodiesterase-5-inhibitor sildenafil and acetyl-cysteine monotherapy. In contrast to the international guideline antacid therapy as a general treatment for IPF was rated negative, based on conflicting results of recent analyses (recommendation C; evidence 4). An unanimous positive recommendation was granted for the antifibrotic drugs nintedanib and pirfenidone for the treatment of IPF (A, 1-a). For some open questions in the management of IPF patients for which firm evidence is lacking the guideline also offers recommendations based on expert consensus.
Insights
New guidelines recommend antifibrotic drugs nintedanib and pirfenidone for idiopathic pulmonary fibrosis (IPF). Several other therapies are not suitable for IPF treatment, based on updated evidence classifications.
Area of Science:
- Pulmonology
- Pharmacology
- Evidence-based medicine
Background:
- Idiopathic pulmonary fibrosis (IPF) is a severe, often fatal lung disease with a median survival of 2-4 years.
- Recent treatment trials necessitate an update to the 2013 German IPF guideline for pharmacological therapies.
Purpose of the Study:
- To update the pharmacological therapy recommendations for IPF based on new evidence.
- To implement the Oxford evidence classification system for a more nuanced assessment of treatments.
Main Methods:
- The Oxford evidence classification system was used, replacing the previous GRADE system.
- Pharmacological therapies were evaluated based on available clinical trial data and expert consensus.
Main Results:
- Nintedanib and pirfenidone received a unanimous positive recommendation (A, 1-a) as antifibrotic drugs for IPF.
- Triple therapy (prednisolone, azathioprine, acetyl-cysteine), imatinib, ambrisentan, bosentan, and macitentan were rated unsuitable (A, 1-b).
- Sildenafil and acetyl-cysteine monotherapy received a negative recommendation (B, 1-b), and antacid therapy was also rated negatively (C, 4).
Conclusions:
- Antifibrotic agents nintedanib and pirfenidone are strongly recommended for IPF treatment.
- Several previously considered or utilized therapies are now deemed unsuitable or have negative recommendations.
- The updated guideline provides expert consensus-based recommendations for areas lacking firm evidence.
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