Small airways pathology in idiopathic pulmonary fibrosis: a retrospective cohort study

Verleden, Stijn E.; Tanabe, Naoya; McDonough, John E.; Vasilescu, Dragoş M.; Xu, Feng; Wuyts, Wim; Piloni, Davide; Sadeleer, Laurens J. De; Willems, Stijn; Mai, Cindy; Hostens, Jeroen; Cooper, Joel D.; Verbeken, Erik; Verschakelen, Johny; Galbán, Craig J.; Raemdonck, Dirk Van; Colby, Thomas V.; Decramer, Marc; Verleden, Geert M.; Kaminski, Naftali; Hackett, Tillie L.; Vanaudenaerde, Bart M.; Hogg, James C.

doi:10.1016/s2213-2600(19)30356-x

Cited by 87 publications

(77 citation statements)

References 33 publications

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“…The sequence of events is completed by the observation of airway wall thickening, and the presence of airway exudates that may contribute to their definitive plugging, precipitating their loss. Again recalling what has been shown in COPD and more recently in IPF [4], but involving completely distinct mechanisms, these lesions are match well with parenchymal destruction, suggesting here again that the first may precede the second. Because together is always better than alone, the reviewing process fed ideas for adding analysis from complimentary scanning electron microscopy.…”

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confidence: 81%

LAM is another small airway disease: lessons from microCT

et al. 2020

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supporting

confidence: 81%

LAM is another small airway disease: lessons from microCT

et al. 2020

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“…Moreover, the emergence of an aberrant airway basal cell population has been widely acknowledged [ 43 , 44 ], as well as reduced angiogenesis [ 45 ]. The early involvement of small airways has recently been discovered [ 46 ], and the important role of airway malformation and development of honeycombing [ 47 ] has been widely adopted, as well as the enigmatic influence of MUC5B polymorphisms and the resulting dysfunctional mucociliary clearance [ 48 , 49 , 50 ]. Recently, it has been suggested that microbiome changes might prove to be an important factor [ 51 , 52 ], irrespective of baseline disease severity [ 53 ].…”

Section: What Makes the Progressive Phenotype Progressive? The Quementioning

confidence: 99%

Towards the Essence of Progressiveness: Bringing Progressive Fibrosing Interstitial Lung Disease (PF-ILD) to the Next Stage

Sadeleer

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Yserbyt

et al. 2020

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Although only recently introduced in the ILD community, the concept of progressive fibrosing interstitial lung disease (PF-ILD) has rapidly acquired an important place in the management of non-idiopathic pulmonary fibrosis fibrosing ILD (nonIPF fILD) patients. It confirms a clinical gut feeling that an important subgroup of nonIPF fILD portends a dismal prognosis despite therapeutically addressing the alleged triggering event. Due to several recently published landmark papers showing a treatment benefit with currently available antifibrotic drugs in PF-ILD patients, endorsing a PF-ILD phenotype has vital therapeutic consequences. Importantly, defining progressiveness is based on former progression, which has proven to be a rather moderate predictor of future progression. As fibrosis extent >20% and the presence of honeycombing have superior predictive properties regarding future progression, we advocate immediate initiation of antifibrotic treatment in the presence of these risk factors. In this perspective, we describe the historical context wherein PF-ILD has emerged, determine the currently employed PF-ILD criteria and their inherent limitations and propose new directions to mature its definition. Finally, while ascertaining progression in a nonIPF fILD patient clearly demonstrates the need for (additional) therapy, in the future, therapeutic decisions should be taken after assessing which pathway is ultimately driving the progression. Although not readily available, pathophysiological insight and diagnostic means are emergent to go full steam ahead in this novel direction.

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“…Those areas without fibrosis on CT show small areas of increased attenuation on µCT, possibly reflecting fibroblast foci [ 30 ]. Interestingly, small airways were equally lost in samples characterized as minimally fibrotic or severely fibrotic on CT, indicating that small airway loss could also be an important process in restrictive lung disease [ 31 ]. The implementation of frozen µCT has advanced the field even more, as structural and morphological information derived from µCT could be combined with biological studies [ 32 ].…”

Section: Imaging and Pathology For Diagnosis Of Lung Fibrosis In Cmentioning

confidence: 99%

From Mouse to Man and Back: Closing the Correlation Gap between Imaging and Histopathology for Lung Diseases

et al. 2020

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Lung diseases such as fibrosis, asthma, cystic fibrosis, infection and cancer are life-threatening conditions that slowly deteriorate quality of life and for which our diagnostic power is high, but our knowledge on etiology and/or effective treatment options still contains important gaps. In the context of day-to-day practice, clinical and preclinical studies, clinicians and basic researchers team up and continuously strive to increase insights into lung disease progression, diagnostic and treatment options. To unravel disease processes and to test novel therapeutic approaches, investigators typically rely on end-stage procedures such as serum analysis, cyto-/chemokine profiles and selective tissue histology from animal models. These techniques are useful but provide only a snapshot of disease processes that are essentially dynamic in time and space. Technology allowing evaluation of live animals repeatedly is indispensable to gain a better insight into the dynamics of lung disease progression and treatment effects. Computed tomography (CT) is a clinical diagnostic imaging technique that can have enormous benefits in a research context too. Yet, the implementation of imaging techniques in laboratories lags behind. In this review we want to showcase the integrated approaches and novel developments in imaging, lung functional testing and pathological techniques that are used to assess, diagnose, quantify and treat lung disease and that may be employed in research on patients and animals. Imaging approaches result in often novel anatomical and functional biomarkers, resulting in many advantages, such as better insight in disease progression and a reduction in the numbers of animals necessary. We here showcase integrated assessment of lung disease with imaging and histopathological technologies, applied to the example of lung fibrosis. Better integration of clinical and preclinical imaging technologies with pathology will ultimately result in improved clinical translation of (therapy) study results.

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Small airways pathology in idiopathic pulmonary fibrosis: a retrospective cohort study

Cited by 87 publications

References 33 publications

LAM is another small airway disease: lessons from microCT

LAM is another small airway disease: lessons from microCT

Towards the Essence of Progressiveness: Bringing Progressive Fibrosing Interstitial Lung Disease (PF-ILD) to the Next Stage

From Mouse to Man and Back: Closing the Correlation Gap between Imaging and Histopathology for Lung Diseases

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