Acute exacerbations of idiopathic pulmonary fibrosis.
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 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.
Collard
Harold R
HR
Department of Medicine, San Francisco General Hospital, University of California, USA.
Moore
Bethany B
BB
Flaherty
Kevin R
KR
Brown
Kevin K
KK
Kaner
Robert J
RJ
King
Talmadge E
TE
Lasky
Joseph A
JA
Loyd
James E
JE
Noth
Imre
I
Olman
Mitchell A
MA
Raghu
Ganesh
G
Roman
Jesse
J
Ryu
Jay H
JH
Zisman
David A
DA
Hunninghake
Gary W
GW
Colby
Thomas V
TV
Egan
Jim J
JJ
Hansell
David M
DM
Johkoh
Takeshi
T
Kaminski
Naftali
N
Kim
Dong Soon
DS
Kondoh
Yasuhiro
Y
Lynch
David A
DA
Müller-Quernheim
Joachim
J
Myers
Jeffrey L
JL
Nicholson
Andrew G
AG
Selman
Moisés
M
Toews
Galen B
GB
Wells
Athol U
AU
Martinez
Fernando J
FJ
D000208:Acute Disease; D006801:Humans; D008168:Lung; D011658:Pulmonary Fibrosis / Q000175:diagnosis / Q000209:etiology / Q000473:pathology / Q000503:physiopathology; D012307:Risk Factors