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Acute exacerbations of idiopathic pulmonary fibrosis Collard HR; Moore BB; Flaherty KR; Brown KK; Kaner RJ; King TE Jr; Lasky JA; Loyd JE; Noth I; Olman MA; Raghu G; Roman J; Ryu JH; Zisman DA; Hunninghake GW; Colby TV; Egan JJ; Hansell DM; Johkoh T; Kaminski N; Kim DS; Kondoh Y; Lynch DA; Muller-Quernheim J; Myers JL; Nicholson AG; Selman M; Toews GB; Wells AU; Martinez FJAm J Respir Crit Care Med 2007[Oct]; 176 (7): 636-43The 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 IPF. 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.|*Pulmonary Fibrosis/diagnosis/etiology/pathology/physiopathology[MESH]|Acute Disease[MESH]|Humans[MESH]|Lung[MESH]|Risk Factors[MESH] |
