Diffuse Pulmonary Ossification in Fibrosing Interstitial Lung Diseases: Prevalence and Associations

Egashira, Ryoko; Jacob, Joseph; Kokosi, Maria; Brun, Anne-Laure; Rice, Alexandra; Nicholson, Andrew G.; Wells, Athol U.; Hansell, David M.

doi:10.1148/radiol.2017152419

Cited by 71 publications

(66 citation statements)

References 36 publications

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“…Some biological hypotheses of DPO pathogenesis have been proposed. DPO is strongly associated with fibrosing interstitial lung diseases ( 24 ); however, the pathogenesis of idiopathic DPO is still uncertain. As a candidate, transforming growth factor-beta (TGF-β)/bone morphogenetic protein (BMP) is known to be associated with ectopic bone formation, as well as normal bone organogenesis ( 25 ).…”

Section: Discussionmentioning

confidence: 99%

Progressive Restrictive Ventilatory Impairment in Idiopathic Diffuse Pulmonary Ossification

et al. 2018

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Diffuse pulmonary ossification (DPO) is a rare disease characterized by metaplastic bone formation in the lung. There are few reports with a long-term follow-up of this disease. We herein report a 47-year-old man diagnosed with idiopathic DPO at 30 years of age. The patient's vital capacity was normal until 36 years of age (3.39 L, 82.4% predicted), but it was severely decreased when he visited the hospital again at 47 years of age due to cough and dyspnea (1.98 L, 44.6% predicted). Chest computed tomography showed a significant increase in the number of high-density nodules, suggesting that the progression of DPO had caused restrictive ventilatory impairment.

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Section: Discussionmentioning

confidence: 99%

Progressive Restrictive Ventilatory Impairment in Idiopathic Diffuse Pulmonary Ossification

et al. 2018

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“…Sie wird in der Regel von einer feinen Retikulation überlagert. In seltenen Fällen können kleine ossifizierte Rundherde in Bereichen der Fibrose vorhanden sein, die bei Patienten mit UIP im Vergleich zu anderen fibrotischen Lungenerkrankungen häufiger auftreten (29 %) [55,56]. Patienten mit UIP-Muster können zusätzlich radiologisch das Muster der pleuroparenchymalen Fibroelastose an den Lungenspitzen aufweisen [57].…”

Section: Uip-musterunclassified

S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose

Behr,

Günther,

Bonella

et al. 2020

Pneumologie

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ZusammenfassungDie idiopathische Lungenfibrose (IPF) ist eine schwerwiegende, chronische Lungenerkrankung, deren Diagnosestellung eine profunde fachliche Expertise verlangt. Seit Veröffentlichung der internationalen IPF-Leitlinie im Jahr 2011 mit Update 2018 haben zahlreiche Studien und technische Fortschritte eine Neubewertung des diagnostischen Vorgehens notwendig gemacht. Angesichts der Indikation, symptomatischen Patienten eine antifibrotische Therapie sofort nach Diagnosestellung zu empfehlen, hat die aktuelle IPF-Diagnostik-Leitlinie zum Ziel, die Diagnose der IPF möglichst ohne Verzögerung, wenig invasiv und zuverlässig zu ermöglichen. Sie beschreibt den typischen klinischen Kontext der IPF sowie die Untersuchungen, die zum Ausschluss bekannter Ursachen einer Lungenfibrose erforderlich sind, einschließlich standardisierter Anamneseerhebung, serologischer Testungen und zellulärer Analyse der bronchoalveolären Lavage. Zentrale Bedeutung kommt dem hochauflösenden Computertomogramm in Dünnschichttechnik zu. Sofern eine Histologie zur Diagnoseeingrenzung erforderlich ist, soll diese in erster Linie mittels der transbronchialen Lungen-Kryobiopsie gewonnen werden. Die chirurgische Lungenbiopsie soll Patienten vorbehalten bleiben, die in ausreichender Verfassung für diesen Eingriff sind und bei denen die endoskopische Diagnostik keine Klärung der Diagnose ergab. Goldstandard ist auch weiterhin die interdisziplinäre Diskussion der erhobenen Befunde, um nach Ausschluss anderer Formen chronisch progredienter Lungenfibrosen die Diagnose der IPF zu stellen.

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“…Moreover, an aberrant deposition of extracellular matrix (ECM) constituents, such as glycosaminoglycans, is characteristic of IPF [ 65 ]. Furthermore, diffuse pulmonary ossification (DPO), i.e., calcification in a collagen matrix leading to bone tissue formation, in the lung parenchyma is common in patients with fibrosing ILD, especially with IPF [ 66 , 67 ].…”

Section: Discussionmentioning

confidence: 99%

Effect of Substrate Stiffness on Physicochemical Properties of Normal and Fibrotic Lung Fibroblasts

et al. 2020

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The presented research aims to verify whether physicochemical properties of lung fibroblasts, modified by substrate stiffness, can be used to discriminate between normal and fibrotic cells from idiopathic pulmonary fibrosis (IPF). The impact of polydimethylsiloxane (PDMS) substrate stiffness on the physicochemical properties of normal (LL24) and IPF-derived lung fibroblasts (LL97A) was examined in detail. The growth and elasticity of cells were assessed using fluorescence microscopy and atomic force microscopy working in force spectroscopy mode, respectively. The number of fibroblasts, as well as their shape and the arrangement, strongly depends on the mechanical properties of the substrate. Moreover, normal fibroblasts remain more rigid as compared to their fibrotic counterparts, which may indicate the impairments of IPF-derived fibroblasts induced by the fibrosis process. The chemical properties of normal and IPF-derived lung fibroblasts inspected using time-of-flight secondary ion mass spectrometry, and analyzed complexly with principal component analysis (PCA), show a significant difference in the distribution of cholesterol and phospholipids. Based on the observed distinctions between healthy and fibrotic cells, the mechanical properties of cells may serve as prospective diagnostic biomarkers enabling fast and reliable identification of idiopathic pulmonary fibrosis (IPF).

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Diffuse Pulmonary Ossification in Fibrosing Interstitial Lung Diseases: Prevalence and Associations

Cited by 71 publications

References 36 publications

Progressive Restrictive Ventilatory Impairment in Idiopathic Diffuse Pulmonary Ossification

Progressive Restrictive Ventilatory Impairment in Idiopathic Diffuse Pulmonary Ossification

S2K-Leitlinie zur Diagnostik der idiopathischen Lungenfibrose

Effect of Substrate Stiffness on Physicochemical Properties of Normal and Fibrotic Lung Fibroblasts

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