Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?

Wuyts, Wim; Cavazza, Alberto; Rossi, Giulio; Bonella, Francesco; Sverzellati, Nicola; Spagnolo, Paolo

doi:10.1183/09059180.00004914

Cited by 109 publications

(92 citation statements)

References 85 publications

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“…It is still unclear what the different pathological patterns found in cases of CHP mean in terms of prognosis, and whether CHP with a UIP-like pattern behaves differently from idiopathic UIP [33]. In this case, MDT discussion and follow-up HRCT did eventually enable a more confident diagnosis of IPF [34]. However, at this stage, the potential benefit of pirfenidone may have been limited by the ongoing progression and severity of disease.…”

Section: Discussionmentioning

confidence: 88%

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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Section: Discussionmentioning

confidence: 88%

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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“…lung interstitium and synovium). This mechanism probably reflects what occurs in RA UIP pattern, and also explains why the UIP pattern of RA ILD has in some cases inflammatory infiltrates 19 .…”

Section: Mechanisms Behind the Concomitance Of Lung And Joint Involvementioning

confidence: 99%

“…Regardless of the specific histology subtype (UIP, NSIP, etc. ), the lung tissue usually shows lymphoid aggregates and even large accumulations of lymphocytes that have the appearance of lymphoid follicles with germinal centers 19,27 . Plasma cells and dendritic cells are also present, but to a lesser amount.…”

Section: Extrapolating From the Treatment Of Other Manifestations Of mentioning

confidence: 99%

Practical Approach to the Evaluation and Management of Rheumatoid Arthritis-Interstitial Lung Disease based on its Proven and Hypothetical Mechanisms

Paulín

Babini

Mamani

et al. 2017

RIC

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The prevalence of interstitial lung disease in patients with rheumatoid arthritis varies from 10 to 42%. Rheumatoid arthritis patients with interstitial lung disease have three times the risk of death compared with those without the disease. Prognosis seems to be related to the high-resolution computed tomography pattern. Usual interstitial pneumonia pattern, resembling idiopathic pulmonary fibrosis, carries a worse prognosis. Validated strategies to identify different phenotypes and assess the disease activity in rheumatoid arthritis interstitial lung disease are lacking. However, the utilization of high-resolution computed tomography, composed disease activity scores, and anti-citrullinated peptide antibodies titers can help to guide decisions in clinical practice. Mechanisms involved in lung disease may be different from those implicated in joint involvement. This could explain why in a significant proportion of cases, interstitial lung disease does not improve or even worsens with standard therapies used successfully to treat the joint component (e.g. anti-tumor necrosis factor agents). In this scenario, a group of drugs that targets the adaptive immune response (e.g. rituximab or abatacept) seems to target more specifically the process that takes place in the lungs. Moreover, the recent emergence of anti-fibrotic drugs, which have already proven effective in idiopathic pulmonary fibrosis, may provide an alternative treatment strategy in rheumatoid arthritis-usual interstitial pneumonia. In this review, we propose a practical approach to the evaluation and therapy of rheumatoid arthritis interstitial lung disease. Validation of strategies directed to assess the activity of lung disease and identify the underlying mechanisms are needed. Clinical trials evaluating a therapeutic approach with specific targets based on the disease phenotype are warranted.

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“…However, it is necessary to underscore that the UIP pattern may also be found in a variety of conditions other than IPF, e.g. connective tissue diseases like rheumatoid arthritis, chronic hypersensitivity pneumonia, and asbestosis [5,6]. The patients with a definite IPF diagnosis could be treated with pirfenidone or nintedanib, the currently 2 approved medications [7,8].…”

Section: Introductionmentioning

confidence: 99%

Idiopathic Pulmonary Fibrosis: Molecular Endotypes of Fibrosis Stratifying Existing and Emerging Therapies

Magnini¹,

Montemurro²,

Iovene³

et al. 2017

Respiration

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Idiopathic pulmonary fibrosis (IPF) is a specific form of chronic, progressive fibrosing interstitial pneumonia of unknown causes. Current diagnostic criteria are based on radiological, clinical, and histopathological features but, unfortunately, still many patients remain undiagnosed. Two currently approved therapies, pirfenidone and nintedanib, slow down disease progression but failed to block or revert it. On the other hand, many of the therapeutic agents tested in several clinical trials have not given satisfactory answers, probably due to the pathological heterogeneity of the disease. A growing number of studies show that IPF phenotype is the common clinical outcome of a variety of different pathophysiological mechanisms that identify disease subgroups characterised by specific genetic and molecular biomarkers (endotypes). The precision medicine approach is identifying and analysing the complex system of genetic, molecular, environmental, and behavioural variables underlying the development of the disease and the response to therapy. These molecular pathways are potential targets for novel agents and useful diagnostic, prognostic, and theragnostic biomarkers. We outline the status of knowledge in this field by discussing the complex pathogenetic pathways underlying different disease subgroups and assessing a stratification approach to novel therapeutic agents based on these endotypes.

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Differential diagnosis of usual interstitial pneumonia: when is it truly idiopathic?

Cited by 109 publications

References 85 publications

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

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

Practical Approach to the Evaluation and Management of Rheumatoid Arthritis-Interstitial Lung Disease based on its Proven and Hypothetical Mechanisms

Idiopathic Pulmonary Fibrosis: Molecular Endotypes of Fibrosis Stratifying Existing and Emerging Therapies

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