Idiopathic pulmonaryfibrosis/cryptogenic fibrosing alveolitis |
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| Authors: | J.?M.?Fellrath,R.?M.?du?Bois mailto:r.dubois@rbh.nthames.nhs.uk" title=" r.dubois@rbh.nthames.nhs.uk" itemprop=" email" data-track=" click" data-track-action=" Email author" data-track-label=" " >Email author |
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| Affiliation: | (1) Division of Respiratory Medicine, Hôpital Universitaire Genève, Geneva, Switzerland;(2) Department of Interstitial Lung Disease, Royal Brompton Hospital, Manresa Road Emmanuel Kaye Building, London swb blr, UK |
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| Abstract: | ![]()
Abstract. Idiopathic pulmonary fibrosis (IPF), synonymous withcryptogenic fibrosing alveolitis (CFA), is a progressive andusually fatal disease of unknown cause characterized bysequential acute lung injury with subsequent scarring andend-stage lung disease. Historically, IPF/CFA encompassed aheterogeneous group of different histological and clinicalentities arising in an idiopathic setting. Recently, theAmerican Thoracic Society (ATS) and European Respiratory Society(ERS) core committee has redefined diagnostic criteria for bothIPF/CFAand idiopathic interstitial pneumonias confining the termIPF/CFA to patients with a histological pattern of usualinterstitial pneumonia on lung biopsy. This review attempts torefine the clinico-radiological-pathological features thattogether define IPF/CFA as it is understood today, and tosummarize the rationale of new therapeutic approaches based onthe current understanding of the pathogenetic mechanisms. |
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| Keywords: | Idiopathic pulmonary fibrosis Cryptogenic fibrosing alveolitis Usual interstitial pneumonia |
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