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A Journal on Diseases of the Respiratory System

Official Journal of the Italian Society of Thoracic Endoscopy
Indexed/Abstracted in: EMBASE, Scopus, Emerging Sources Citation Index




Minerva Pneumologica 2010 March;49(1):73-82

language: English

Management of idiopathic pulmonary fibrosis

Amlani S. 1, Richeldi L. 2, Kolb M. R. J. 1,3

1 Department of Medicine, McMaster University, St. Joseph’s Healthcare, Firestone Institute for Respiratory Health, Hamilton, ON, Canada
2 Center for Rare Lung Disease (MaRP), University of Modena and Reggio Emilia, Modena, Italy
3 Department of Pathology and Molecular Medicine, Center for Gene Therapeutics, Hamilton, ON, Canada


Idiopathic pulmonary fibrosis (IPF) is a debilitating progressive respiratory disease with poor prognosis. The clinical management of IPF is difficult and no efficacious therapies are available. Advances in radiographic technique have made HRCT to a reliable, highly specific clinical tool that allows establishing the diagnosis in a majority of cases without the need for surgical biopsy. Recent research has shown that IPF, initially thought to be a chronic inflammatory disorder, is likely the result of aberrant wound healing. Many new treatment targets have been identified and novel compounds hold future promise for patients with the disease. This review summarizes the key features of IPF, both well known characteristics and recent advances in research, which will help in the management of patients in daily practice.

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