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Diagnosis of Idiopathic Pulmonary Fibrosis: Differential Diagnosis
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Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes

Servei Pneumologia, Hospital Universitari Son Espases, 07010 Palma Mallorca, Spain
Institut de Investigacio Sanitària de les Illes Balears (IdISBa), 07120 Palma Mallorca, Spain
CIBER Enfermedades Respiratorias, Insituto Carlos III, 28029 Madrid, Spain
Instituto de Investigación, Hospital Universitario de la Princesa (IISP), Universidad Autónoma de Madrid, 28006 Madrid, Spain
Author to whom correspondence should be addressed.
Scientific and Methodological Consultant of SEPAR.
Med. Sci. 2018, 6(4), 110;
Received: 7 September 2018 / Revised: 16 November 2018 / Accepted: 21 November 2018 / Published: 29 November 2018
(This article belongs to the Special Issue Idiopathic Pulmonary Fibrosis)
Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is characterized by a chronic, progressive, fibrotic interstitial lung disease of unknown cause that occurs primarily in older adults. Its prevalence and incidence have appeared to be increasing over the last decades. Despite its unknown nature, several genetic and environmental factors have been associated with IPF. Moreover, its natural history is variable, but could change depending on the currently suggested phenotypes: rapidly progressive IPF, familial, combined pulmonary fibrosis and emphysema, pulmonary hypertension, and that associated with connective tissue diseases. Early recognition and accurate staging are likely to improve outcomes and induce a prompt initiation of antifibrotics therapy. Treatment is expected to be more effective in the early stages of the disease, while developments in treatment aim to improve the current median survival of 3–4 years after diagnosis. View Full-Text
Keywords: idiopathic pulmonary fibrosis; prevalence; incidence; evolution; risk factors; comorbidities idiopathic pulmonary fibrosis; prevalence; incidence; evolution; risk factors; comorbidities
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Sauleda, J.; Núñez, B.; Sala, E.; Soriano, J.B. Idiopathic Pulmonary Fibrosis: Epidemiology, Natural History, Phenotypes. Med. Sci. 2018, 6, 110.

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