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Respiration. 2019;97(2):173-184. doi: 10.1159/000495046. Epub 2018 Dec 13.

Nintedanib in Idiopathic Pulmonary Fibrosis: Practical Management Recommendations for Potential Adverse Events.

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Department of Respiratory Diseases and Allergy, Aarhus University Hospital, Aarhus, Denmark,
Department of Respiratory Diseases, Interstitial Lung Disease and Lung Transplant Unit, University Hospitals Leuven, Leuven, Belgium.
KU Leuven, Department of Clinical and Experimental Medicine, Division of Respiratory Diseases, Laboratory of Respiratory Diseases, Lung Transplantation Unit, Leuven, Belgium.
Pneumology Unit A, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal.
North West Interstitial Lung Disease Unit, Manchester University Foundation Trust, Wythenshawe, Manchester, United Kingdom.
Department of Pulmonary Medicine, Erasmus Medical Center Cancer Institute, Rotterdam, The Netherlands.
Center for interstitial and rare lung diseases, Pneumology and respiratory critical care medicine, Thoraxklinik, University of Heidelberg, and Translational Lung Research Center Heidelberg, Member of the German Center for Lung Research (DZL), Heidelberg, Germany.
Department of Cardiology, Aarhus University Hospital, Aarhus N, Denmark.
Unit of Thrombosis Research, Hospital of Southwest, University of Southern Denmark, Esbjerg, Denmark.
University of Helsinki and Helsinki University Hospital, Heart and Lung Center, Department of Pulmonary Medicine, Helsinki, Finland.
Department of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, Italy.
Translational Research Center for Gastrointestinal Disorders (TARGID) and Department of Gastroenterology, University of Leuven, Leuven, Belgium.
Department of Pulmonary Disease, Erasmus Medical Centre, University Hospital Rotterdam, Rotterdam, The Netherlands.


Idiopathic pulmonary fibrosis (IPF) is a fatal lung disease with a dismal survival rate of only 3 years and no curative pharmacological therapy. The recent approval of 2 anti-fibrotic drugs (nintedanib and pirfenidone) that slow disease progression has provided some hope for patients. However, effectively managing anti-fibrotic treatment can be a challenge due to tolerability issues, the presence of pulmonary and extra-pulmonary comorbidities, and the need for concomitant medications in many patients. In general, making clear evidence-based decisions can be difficult for physicians because patients with comorbidities are often excluded from clinical trials. Since currently anti-fibrotic drugs are the only effective therapeutics capable of slowing disease progression, it is imperative that all treatment options are thoroughly evaluated and exhausted in each individual, irrespective of complicating factors, to permit the best outcome for the patient. In this review, we present data from clinical trials, post hoc analyses, post-marketing surveillance, and real-world studies that are relevant to the management of nintedanib treatment. In addition, we also provide practical recommendations developed by a multidisciplinary panel of experts for the management of nintedanib treatment in patients with IPF associated complications and those experiencing gastrointestinal side effects.


Expert opinion; Idiopathic pulmonary fibrosis; Nintedanib; Treatment


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