Abstract
The incidence and prevalence of idiopathic pulmonary fibrosis (IPF) is increasing
worldwide. This, combined with its poor prognosis and unpredictable natural history,
has amplified the importance of an accurate diagnosis and monitoring. A diagnosis
of exclusion, IPF requires a comprehensive clinical evaluation. This results in a
clinical context that provides the backdrop for interpretation of the chest imaging
and histopathology. A confident or probable usual interstitial pneumonia chest imaging
pattern on high-resolution computerized tomography may be diagnostic in the correct
clinical context. Outcomes for IPF are unpredictable, ranging from rapid progression
with death within months to prolonged stability. Disease activity is monitored by
clinical and physiology measures with a declining forced vital capacity, a recognized
measure of progression. The available treatments, pirfenidone and nintedanib, are
effective at reducing the expected decline in forced vital capacity.
Key Indexing Terms
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Article info
Publication history
Published online: February 15, 2019
Accepted:
February 13,
2019
Received:
February 13,
2019
Footnotes
Source of Funding: None.
Conflict of Interest: Y.W. reports no conflicts of interest. K.K.B. reports multiple lung fibrosis grants from the National Heart, Lung, and Blood Institute.
Identification
Copyright
© 2019 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.
