Background: This document provides clinical recommendations for the diagnosis of idiopathic pulmonary fibrosis (IPF). It represents a collaborative effort between the American Thoracic Society, Eur...

https://www.atsjournals.org/doi/pdf/10.1164/rccm.201807-1255ST

Diagnosis of Idiopathic Pulmonary Fibrosis. An Official ATS/ERS/JRS/ALAT Clinical Practice Guideline.

Purpose To determine high resolution computed tomography (HRCT) patterns and pulmonary function indices which are associated with increased mortality in patients with connective tissue disease related fibrotic lung disease (CTD-FLD). Methods HRCTs from 168 patients with CTD-FLD were scored by 2 observers for a variety of HRCT patterns and traction bronchiectasis. A radiological diagnosis of usual interstitial pneumonia (UIP), fibrotic non-specific interstitial pneumonia (NSIP) or indeterminate was also assigned. Using Cox regression analysis, associations with mortality were identified. Honeycombing and traction bronchiectasis scores were converted to binary absence/presence scores and also tested. A subgroup analysis of patients with biopsy material (n=51) was performed by classifying patients according to radiological and histopathological diagnoses, as concordant UIP, discordant UIP and fibrotic NSIP. The prognostic separation of this classification was also evaluated. Results Severity of traction bronchiectasis (HR 1.10, p=0.001, 95% CIs 1.04 to 1.17), increasing extent of honeycombing (HR 1.08, p=0.021, 95% CI 1.03 to 1.13) and reduction in DLco (HR 0.97, p=0.013, 95% CI 0.95 to 0.99) were independently associated with increased mortality. Interobserver agreement and prognostic strength were higher for binary traction bronchiectasis scores (weighted κ (κw)=0.69, HR 4.00, p=0.001, 95%CI 1.19 to 13.38), than binary honeycombing scores (κw=0.50, HR 2.87, p=0.022, 95% CI 1.53 to 5.43). The radiological-histopathological classification was strongly associated with increased mortality (HR 2.74, p Conclusions Severity of traction bronchiectasis, extent of honeycombing and DLco are strongly associated with mortality in CTD-FLD. Interobserver agreement for traction bronchiectasis is higher than for honeycombing. In CTD-FLD, radiological diagnosis has survival implications in biopsy proven UIP.

https://thorax.bmj.com/content/thoraxjnl/69/3/216.full.pdf

Connective tissue disease related fibrotic lung disease: high resolution computed tomographic and pulmonary function indices as prognostic determinants

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive fibrosing lung disorder of unknown aetiology whose diagnosis involves the careful exclusion of secondary causes for pulmonary fibrosis and the presence of a pattern of usual interstitial pneumonia (UIP) at either high-resolution computed tomography (HRCT) scan or surgical lung biopsy. Despite great efforts made in establishing precise, universally acknowledged diagnostic criteria for IPF, its ascertainment remains a challenge, especially in those individuals presenting with atypical HRCT patterns. With new drugs emerging, establishing a precise diagnosis is becoming a clinically relevant issue. Although regarded as a rare disease, IPF epidemiology is controversial due to studies relying on old data and adopting mixed, incomparable methodologies for cases definition. Overall, the prevalence and incidence appear to be increasing over the last decades, suggesting that in earlier studies they might have been underestimated because of diagnostic uncertainty. IPF is invariably progressive, although its clinical course might greatly vary on an individual basis, with episodes of severe acute respiratory deterioration (acute exacerbations) being unpredictable. A deeper understanding of the mechanisms responsible for an accelerated course of the disease and the identification of biomarkers of progression would lead to a better stratification of the disease, essential for delivering individualized therapeutic strategies.

https://onlinelibrary.wiley.com/doi/pdf/10.1111/resp.12683

Idiopathic pulmonary fibrosis: Diagnosis, epidemiology and natural history

Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease with great variability in disease severity and rate of progression. The need for a reliable, sensitive, and objective biomarker to track disease progression and response to therapy remains a great challenge in IPF clinical trials. Over the past decade, quantitative computed tomography (QCT) has emerged as an area of intensive research to address this need. We have gathered a group of pulmonologists, radiologists and scientists with expertise in this area to define the current status and future promise of this imaging technique in the evaluation and management of IPF. In this Pulmonary Perspective, we review the development and validation of six computer-based QCT methods and offer insight into the optimal use of an imaging-based biomarker as a tool for prognostication, prediction of response to therapy, and potential surrogate endpoint in future therapeutic trials.

/pdf/computed-tomographic-biomarkers-in-idiopathic-pulmonary-3mjbvxq2ng.pdf

Computed Tomographic Biomarkers in Idiopathic Pulmonary Fibrosis. The Future of Quantitative Analysis.

Idiopathic pulmonary fibrosis (IPF), the most prevalent idiopathic interstitial pneumonia, is associated with a poor prognosis. An accurate diagnosis of IPF is essential for optimal management. The recent ATS/ERS/JRS/ALAT recommendations on the diagnosis and management of IPF were developed from a systematic review of the published literature. High-resolution computed tomography (HRCT) scanning has a central role in the IPF diagnostic pathway with formal designation of criteria for an HRCT pattern of UIP. In the correct clinical context, a UIP pattern on HRCT is indicative of a definite diagnosis of IPF without the need for a surgical lung biopsy. However, although the 2011 ATS/ERS/JRS/ALAT statement is a major advance, the application of guideline recommendations by clinicians has identified limitations that need to be addressed in future statements. Key problems include: 1) the lack of management recommendations for the highly prevalent clinical scenarios of probable and possible IPF; 2) the ongoing confusion about the diagnostic role of bronchoalveolar lavage (reflecting ambiguity in the current recommendation); 3) HRCT misdiagnosis by less experienced radiologists, increasingly recognised as a major problem; and 4) the lack of integration of clinical data, including the treated course of disease, in the designation of the diagnostic likelihood of IPF.

/pdf/the-revised-ats-ers-jrs-alat-diagnostic-criteria-for-4n17gdw22w.pdf

The revised ATS/ERS/JRS/ALAT diagnostic criteria for idiopathic pulmonary fibrosis (IPF) - practical implications

OBJECTIVE. The purpose of this study was to determine whether the predominant findings at high-resolution CT influence the accuracy of diagnosis of diffuse lung disease.MATERIALS AND METHODS. The cases of 100 patients with diffuse lung disease who underwent high-resolution CT and tissue diagnosis were studied. Three thoracic radiologists reviewed high-resolution CT images blindly and independently for patterns of abnormality, listing their three main diagnoses and level of confidence in the first choice. The effect of the findings on accuracy was analyzed.RESULTS. For honeycombing, the accuracy of the main diagnosis was 96.6%, 92.2%, and 92.3% for the three readers, and that of the three main diagnoses was 96.6%, 96.1%, and 92.3%. For cysts, the accuracy of the main diagnosis was 88.9%, 80%, and 81.8% and of the three main diagnoses was 100%, 90%, and 90.9%. For bronchovascular thickening, the accuracy of the main diagnosis was 91.7%, 87.5%, and 90.9% and of the three main diagnoses was 91.7%, 100%, and 9...

https://www.ajronline.org/doi/pdfplus/10.2214/AJR.07.3177

Accuracy of high-resolution CT in the diagnosis of diffuse lung disease: effect of predominance and distribution of findings.

Purpose The purpose of this work was to illustrate the CT appearance of the independent origin of the hepatic and splenic arteries from the abdominal aorta. Method A retrospective review of available scans from 159 consecutive contrast-enhanced abdominal CT examinations was undertaken to document the frequency with which this vascular variant is seen at helical CT. Results Independent origin of the hepatic and splenic arteries was seen in 2 of 159 examinations (1.3%). This correlates well with the 1% incidence reported in the angiographic and surgical literature. Conclusion Independent origin of the hepatic and splenic arteries from the aorta should be seen in approximately 1% of abdominal CT examinations. Knowledge of this vascular variant and its CT appearance may prevent confusion or mislabeling.

Independent origin of the hepatic and splenic arteries from the abdominal aorta: CT demonstration

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Accuracy of high-resolution CT in the diagnosis of diffuse lung disease: effect of predominance and distribution of findings.

Independent origin of the hepatic and splenic arteries from the abdominal aorta: CT demonstration