Comorbidities and Complications in Idiopathic Pulmonary Fibrosis

Cano-Jiménez, Esteban; González, Fernanda Hernández; Bermudo, Guadalupe

doi:10.3390/medsci6030071

Cited by 18 publications

(18 citation statements)

References 58 publications

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“…Acute exacerbations of IPF are commonly encountered in patients with low Forced Vital Capacity (FVC), Diffusion lung CO (DLCO), low six-minute-walk distance, pulmonary hypertension, poor baseline oxygenation, increased dyspnea and other cardiovascular disease or high body mass index [41], and furthermore, an elevated serum level of Krebs von Lungen-6 (KL-6) [42].…”

Section: Acute Exacerbationmentioning

confidence: 99%

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

et al. 2020

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Idiopathic pulmonary fibrosis (IPF) is a fibrotic lung disease with uncertain origins and pathogenesis; it represents the most common interstitial lung disease (ILD), associated with a pathological pattern of usual interstitial pneumonitis (UIP). This disease has a poor prognosis, having the most lethal prognosis among ILDs. In fact, the progressive fibrosis related to IPF could lead to the development of complications, such as acute exacerbation, lung cancer, infections, pneumothorax and pulmonary hypertension. Pneumologists, radiologists and pathologists play a key role in the identification of IPF disease, and in the characterization of its complications—which unfortunately increase disease mortality and reduce overall survival. The early identification of these complications is very important, and requires an integrated approach among specialists, in order to plane the correct treatment. In some cases, the degree of severity of patients having IPF complications may require a personalized approach, based on palliative care services. Therefore, in this paper, we have focused on clinical and radiological features of the complications that occurred in our IPF patients, providing a comprehensive and accurate pictorial essay for clinicians, radiologists and surgeons involved in their management.

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Section: Acute Exacerbationmentioning

confidence: 99%

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

et al. 2020

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“…In regard to mechanical properties of the lungs however, patients with CPFE have been shown to most often have only mild impairment in spirometric and lung volume indices despite rather severe imaging and gas exchange abnormalities. This observation suggests the abnormal lung mechanics of each individual process are likely being counter-balanced which results in relative preservation of lung volumes and elastic recoil properties [1,2,4,5,6,39].…”

Section: Combined Emphysema and Fibrosismentioning

confidence: 99%

“…The presence of only mild impairment in spirometry along with a preserved FEV 1 /FVC ratio has led to the consideration that the abnormal respiratory mechanics of emphysema are being counterbalanced in some manner by the abnormal respiratory mechanics of pulmonary fibrosis. This observation has been sometimes referred to as pseudo-normalization of spirometry and lung volume parameters in these patients with CPFE [5,6].…”

Section: Introductionmentioning

confidence: 99%

Combined Pulmonary Fibrosis and Emphysema: Pulmonary Function Testing and a Pathophysiology Perspective

et al. 2019

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Combined pulmonary fibrosis and emphysema (CPFE) has been increasingly recognized over the past 10–15 years as a clinical entity characterized by rather severe imaging and gas exchange abnormalities, but often only mild impairment in spirometric and lung volume indices. In this review, we explore the gas exchange and mechanical pathophysiologic abnormalities of pulmonary emphysema, pulmonary fibrosis, and combined emphysema and fibrosis with the goal of understanding how individual pathophysiologic observations in emphysema and fibrosis alone may impact clinical observations on pulmonary function testing (PFT) patterns in patients with CPFE. Lung elastance and lung compliance in patients with CPFE are likely intermediate between those of patients with emphysema and fibrosis alone, suggesting a counter-balancing effect of each individual process. The outcome of combined emphysema and fibrosis results in higher lung volumes overall on PFTs compared to patients with pulmonary fibrosis alone, and the forced expiratory volume in one second (FEV1)/forced vital capacity (FVC) ratio in CPFE patients is generally preserved despite the presence of emphysema on chest computed tomography (CT) imaging. Conversely, there appears to be an additive deleterious effect on gas exchange properties of the lungs, reflecting a loss of normally functioning alveolar capillary units and effective surface area available for gas exchange, and manifested by a uniformly observed severe reduction in the diffusing capacity for carbon monoxide (DLCO). Despite normal or only mildly impaired spirometric and lung volume indices, patients with CPFE are often severely functionally impaired with an overall rather poor prognosis. As chest CT imaging continues to be a frequent imaging modality in patients with cardiopulmonary disease, we expect that patients with a combination of pulmonary emphysema and pulmonary fibrosis will continue to be observed. Understanding the pathophysiology of this combined process and the abnormalities that manifest on PFT testing will likely be helpful to clinicians involved with the care of patients with CPFE.

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“…PAH is the major cause of death in SSc patients, together with pulmonary fibrosis [47]. It can be an isolated tardive complication of lSSc, with anti-centromere antibodies, or a secondary manifestation-related pulmonary fibrosis [48].…”

Section: Systemic Sclerosismentioning

confidence: 99%

Exosomes in Systemic Sclerosis: Messengers Between Immune, Vascular and Fibrotic Components?

Colletti

Galardi

Santis

et al. 2019

IJMS

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Systemic sclerosis (SSc) is a rare autoimmune disease, characterized by vasculopathy and fibrosis of the skin and internal organs. This disease is still considered incurable and is associated with a high risk of mortality, which is related to fibrotic events. An early diagnosis is useful for preventing complications, and targeted therapies reduce disease progression and ameliorate patients’ quality of life. Nevertheless, there are no validated biomarkers for early diagnosis with predictive prognostic value. Exosomes are membrane vesicles, transporting proteins and nucleic acids that may be delivered to target cells, which influences cellular behavior. They play important roles in cell–cell communication, both in physiological and pathological conditions, and may be useful as circulating biomarkers. Recent evidences suggest a role for these microvesicles in the three main aspects related to the pathogenesis of SSc (immunity, vascular damage, and fibrosis). Moreover, exosomes are of particular interest in the field of nano-delivery and are used as biological carriers. In this review, we report the latest information concerning SSc pathogenesis, clinical aspects of SSc, and current approaches to the treatment of SSc. Furthermore, we indicate a possible role of exosomes in SSc pathogenesis and suggest their potential use as diagnostic and prognostic biomarkers, as well as therapeutic tools.

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Comorbidities and Complications in Idiopathic Pulmonary Fibrosis

Cited by 18 publications

References 58 publications

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

Complications in Idiopathic Pulmonary Fibrosis: Focus on Their Clinical and Radiological Features

Combined Pulmonary Fibrosis and Emphysema: Pulmonary Function Testing and a Pathophysiology Perspective

Exosomes in Systemic Sclerosis: Messengers Between Immune, Vascular and Fibrotic Components?

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