Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation

Poletti, Venerino; Ravaglia, Claudia; Buccioli, Matteo; Tantalocco, Paola; Piciucchi, Sara; Dubini, Alessandra; Carloni, Angelo; Chilosi, Marco; Tomassetti, Sara

doi:10.1159/000353580

Cited by 45 publications

(32 citation statements)

References 74 publications

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“…In IPF, progressive lung fibrosis is thought to be related to recurrent injury to the alveolar epithelium followed by aberrant wound healing [5]. Clinically, this manifests as a reduction in both forced vital capacity and diffusing capacity of the lung for carbon monoxide (D LCO ).…”

Section: The Problemmentioning

confidence: 99%

Principles of Rehabilitation and Reactivation: Interstitial Lung Disease, Sarcoidosis and Rheumatoid Disease with Respiratory Involvement

Holland¹,

Dowman

Hill

2015

Respiration

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The interstitial lung diseases (ILDs) are characterised by dyspnoea on exertion, exercise-induced hypoxaemia, reduced skeletal muscle function and exercise intolerance. Evidence from nine randomised controlled trials shows that pulmonary rehabilitation improves exercise capacity, dyspnoea and quality of life in ILD, with moderately large effect sizes from 0.59 to 0.68. Participants with idiopathic pulmonary fibrosis, the most common and most progressive of the ILDs, achieve benefits in exercise capacity and quality of life that are of equal magnitude to those seen in other ILDs, with effect sizes from 0.59 to 0.75. Whole body exercise training is a core component of pulmonary rehabilitation for ILD. The standard exercise prescription used for other chronic lung diseases is effective in ILD, including 8 weeks of training with at least two supervised sessions per week and at least 30 min of aerobic training per session. However, the unique presentation and underlying pathophysiology of ILD may require modifications of the exercise prescription for individual patients. Those with connective tissue disease may present with joint pain and stiffness that require modification of the standard exercise prescription, including reduction in weight-bearing exercise. Some patients with severe disease may present with distressing dyspnoea that limits the intensity or progression of training. Because exercise-induced hypoxaemia is common in ILD and more severe than seen in other chronic lung diseases, pulmonary rehabilitation should be provided in a setting where supplemental oxygen therapy is available. Pulmonary rehabilitation programs offer the opportunity to address other critical aspects of ILD care, including management of comorbidities, symptoms and mood.

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Section: The Problemmentioning

confidence: 99%

Principles of Rehabilitation and Reactivation: Interstitial Lung Disease, Sarcoidosis and Rheumatoid Disease with Respiratory Involvement

Holland¹,

Dowman

Hill

2015

Respiration

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“…Another possible approach to ILD classification and diagnosis is to consider the disease behaviour [10]. However, IPF natural history may vary among individual patients and may follow one of several clinical courses [11][12][13]. The majority of patients have slowly progressive disease, some have an accelerated decline followed by death, and some have periods of relatively stable disease interposed with episodes of acute respiratory worsening.…”

Section: Introductionmentioning

confidence: 99%

The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

Tomassetti¹,

Piciucchi

Tantalocco³

et al. 2015

Eur Respir Rev

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Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressively fibrosing interstitial pneumonia that is associated with a significantly worse prognosis than other forms of chronic interstitial pneumonia. An early and accurate diagnosis of IPF is important to enable the initiation of disease-specific therapies, which have the potential to reduce disease progression, and the avoidance of inappropriate and potentially harmful drugs. Establishing an accurate diagnosis of IPF can be challenging. Recent studies and international guidelines advocate the importance of a multidisciplinary team (MDT) in the initial diagnostic assessment of patients with suspected IPF. Typical MDT members include a pulmonologist, a radiologist and a pathologist, with further input from a thoracic surgeon, a rheumatologist, a specialist nurse and an occupational physician where appropriate. Multidisciplinary diagnosis is considered the gold standard because it can improve the accuracy of diagnosis of IPF, avoid unnecessary testing (e.g. lung biopsy), and optimise patient management. Here we highlight the strengths and limitations of the multidisciplinary approach to IPF diagnosis through MDT discussion of two patient cases. @ERSpublicationsThe strengths and limitations of IPF management by a multidisciplinary team are highlighted in two patient cases http://ow.ly/H04Xe

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“…To date, the majority of available data are from studies performed in the USA and northern Europe [79,80]. PORRETTA et al [81] estimated that annual IPF incidence in Italy ranged between 750 000 and 490 000.…”

Section: Diffuse Parenchymal Lung Diseasesmentioning

confidence: 99%

Clinical highlights from the 2013 ERS Congress in Barcelona

Spruit¹,

Marvisi

Coolen³

et al. 2014

Eur Respir J

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This article reviews a selection of scientific presentations in the area of clinical problems, which were presented at the 2013 European Respiratory Society Annual Congress in Barcelona, Spain. This article discusses the most relevant topics of interest in the field of clinical respiratory medicine, including breakthrough reports and studies of particular interest to the healthcare professionals. Topics are presented and discussed in the context of the most up-to-date literature, including basic science and translational research. In particular, the reviewed topics deal with the areas of complex chronic obstructive pulmonary disease and asthma (even in the primary care setting), idiopathic pulmonary fibrosis (pathogenesis and therapy), advances in functional chest imaging, interventional pulmonology, pulmonary rehabilitation and chronic care.

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Idiopathic Pulmonary Fibrosis: Diagnosis and Prognostic Evaluation

Cited by 45 publications

References 74 publications

Principles of Rehabilitation and Reactivation: Interstitial Lung Disease, Sarcoidosis and Rheumatoid Disease with Respiratory Involvement

Principles of Rehabilitation and Reactivation: Interstitial Lung Disease, Sarcoidosis and Rheumatoid Disease with Respiratory Involvement

The multidisciplinary approach in the diagnosis of idiopathic pulmonary fibrosis: a patient case-based review

Clinical highlights from the 2013 ERS Congress in Barcelona

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