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J Intern Med. 2017 Feb;281(2):149-166. doi: 10.1111/joim.12571. Epub 2016 Nov 13.

Treatment of idiopathic pulmonary fibrosis: a position paper from a Nordic expert group.

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Respiratory Medicine Unit, Center for Molecular Medicine, Department of Medicine, Solna Karolinska Institutet, Stockholm, Sweden.
Lung-Allergy Clinic Karolinska University Hospital Solna, Stockholm, Sweden.
Department of Respiratory Medicine and Allergy, Aarhus University Hospital, Aarhus, Denmark.
Transplantation laboratory and Heart and Lung Center, University of Helsinki and Helsinki University Hospital, Helsinki, Finland.
Department of Respiratory Medicine and Sleep, Faculty of Medicine, Landspitali University Hospital, University of Iceland, Reykjavik, Iceland.
Department of Respiratory Medicine, Oslo University Hospital, Rikshospitalet, Oslo, Norway.
Department of Pulmonary Medicine, University of Tartu, Tartu, Estonia.
Lung Clinic, Tartu University Hospital, Tartu, Estonia.
Unit of Medicine and Clinical Research, Pulmonary Division, Division of Respiratory Medicine, University of Eastern Finland and Center of Medicine and Clinical Research, Kuopio, Finland.
Respiratory research, Research Unit of Internal Medicine, Medical Research Center, University of Oulu and Oulu University Hospital, Oulu, Finland.
Kuopio University Hospital, Kuopio, Finland.


Idiopathic pulmonary fibrosis (IPF) is a fatal progressive lung disease occurring in adults. In the last decade, the results of a number of clinical trials based on the updated disease classification have been published. The registration of pirfenidone and nintedanib, the first two pharmacological treatment options approved for IPF, marks a new chapter in the management of patients with this disease. Other nonpharmacological treatments such as lung transplantation, rehabilitation and palliation have also been shown to be beneficial for these patients. In this review, past and present management is discussed based on a comprehensive literature search. A treatment algorithm is presented based on available evidence and our overall clinical experience. In addition, unmet needs with regard to treatment are highlighted and discussed. We describe the development of various treatment options for IPF from the first consensus to recent guidelines based on evidence from large-scale, multinational, randomized clinical trials, which have led to registration of the first drugs for IPF.


idiopathic interstitial pneumonias; lung transplantation; nintedanib; pirfenidone; pulmonary rehabilitation

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