Journal
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE
Volume 176, Issue 7, Pages 636-643Publisher
AMER THORACIC SOC
DOI: 10.1164/rccm.200703-463PP
Keywords
acute exacerbation; pulmonary fibrosis; diagnosis; definition
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Funding
- NHLBI NIH HHS [HL080383, R01 HL058655, HL6767, U10 HL080411-03, HL083901, HL58655, R01 HL083901, HL86516, U10 HL080383, K23 HL086516] Funding Source: Medline
- PHS HHS [080685] Funding Source: Medline
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The natural history of idiopathic pulmonary fibrosis (IPF) has been characterized as a steady, predictable decline in lung function over time. Recent evidence suggests that some patients may experience a more precipitous course, with periods of relative stability followed by acute deteriorations in respiratory status. Many of these acute deteriorations are of unknown etiology and have been termed acute exacerbations of IPF. This perspective is the result of an international effort to summarize the current state of knowledge regarding acute exacerbations of IPF. Acute exacerbations of IPF are defined as acute, clinically significant deteriorations of unidentifiable cause in patients with underlying IIPF. Proposed diagnostic criteria include subjective worsening over 30 days or less, new bilateral radiographic opacities, and the absence of infection or another identifiable etiology. The potential pathobiological roles of infection, disordered cell biology, coagulation, and genetics are discussed, and future research directions are proposed.
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