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2016 ; 5
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English Wikipedia
Recent advances in understanding idiopathic pulmonary fibrosis
#MMPMID27303645
Daccord C
; Maher TM
F1000Res
2016[]; 5
(ä): ä PMID27303645
show ga
Despite major research efforts leading to the recent approval of pirfenidone and
nintedanib, the dismal prognosis of idiopathic pulmonary fibrosis (IPF) remains
unchanged. The elaboration of international diagnostic criteria and disease
stratification models based on clinical, physiological, radiological, and
histopathological features has improved the accuracy of IPF diagnosis and
prediction of mortality risk. Nevertheless, given the marked heterogeneity in
clinical phenotype and the considerable overlap of IPF with other fibrotic
interstitial lung diseases (ILDs), about 10% of cases of pulmonary fibrosis
remain unclassifiable. Moreover, currently available tools fail to detect early
IPF, predict the highly variable course of the disease, and assess response to
antifibrotic drugs. Recent advances in understanding the multiple interrelated
pathogenic pathways underlying IPF have identified various molecular phenotypes
resulting from complex interactions among genetic, epigenetic, transcriptional,
post-transcriptional, metabolic, and environmental factors. These different
disease endotypes appear to confer variable susceptibility to the condition,
differing risks of rapid progression, and, possibly, altered responses to
therapy. The development and validation of diagnostic and prognostic biomarkers
are necessary to enable a more precise and earlier diagnosis of IPF and to
improve prediction of future disease behaviour. The availability of approved
antifibrotic therapies together with potential new drugs currently under
evaluation also highlights the need for biomarkers able to predict and assess
treatment responsiveness, thereby allowing individualised treatment based on risk
of progression and drug response. This approach of disease stratification and
personalised medicine is already used in the routine management of many cancers
and provides a potential road map for guiding clinical care in IPF.