[Show abstract][Hide abstract] ABSTRACT: Idiopathic pulmonary fibrosis (IPF), the most common and lethal of the idiopathic interstitial pneumonias, is defined by a radiological and/or pathological pattern of usual interstitial pneumonia (UIP). However, UIP is not synonymous with IPF as other clinical conditions may be associated with UIP, including chronic hypersensitivity pneumonitis, collagen vascular disease, drug toxicity, asbestosis, familial IPF and Hermansky-Pudlak syndrome. Differentiating IPF ("idiopathic UIP") from conditions that mimic IPF ("secondary UIP") has substantial therapeutic and prognostic implications. A number of radiological and histological clues may help distinguish IPF from other conditions with a UIP pattern of fibrosis, but their appreciation requires extensive expertise in interstitial lung disease as well as an integrated multidisciplinary approach involving pulmonologists, radiologists and pathologists. In addition, multidisciplinary discussions may decrease the time to initial IPF diagnosis and, thus, enable more timely management. This concept was strongly emphasised by the 2011 ATS/ERS/JRS/ALAT guidelines. This article highlights, with the aid of a clinical case, the difficulties in making a diagnosis of IPF in clinical practice. Yet, an accurate diagnosis is critical, particularly given the availability of drugs that may reduce the pace of functional decline and disease progression in IPF.
European Respiratory Review 09/2014; 23(133):308-319.
[Show abstract][Hide abstract] ABSTRACT: BACKGROUND
Lung cancer (LC) is frequently associated with idiopathic pulmonary fibrosis (IPF), despite this well-known association the outcome of LC in IPF patients is unclear. The objective of this study was to evaluate the impact of LC on survival of patients with associated IPF. METHODSA total of 260 patients with IPF were reviewed and 186 IPF cases had complete clinical and follow-up data. Among these 5 cases were excluded because LC was radiologically suspected but not histologically proven. The remaining 181 cases were categorized in two groups: 23 patients with biopsy proven LC and IPF (LC-IPF) and 158 patients with IPF only (IPF). Survival and clinical characteristics of the two groups were compared. RESULTSPrevalence of histologically proven LC was 13%, and among those with LC-IPF cumulative incidence at 1 and 3 years was 41% and 82%. Patients with LC were more frequently smokers (91.3% vs 71.6%, p= 0.001), with combined pulmonary fibrosis and emphysema (CPFE) (52% vs 32%, p = 0.052). Survival in patients with LC-IPF was significantly worse than IPF patients without LC (median survival 38.7 months versus 63.9 months (HR=5.0; 95% CI: 2.91-8.57; p<0.001). Causes of death in the study group were respiratory failure in 43% of patients, LC progression in 13 % and LC treatment related complications in 17%. CONCLUSION
In patients with IPF LC has a significant adverse impact on survival. Diagnosis and treatment of LC in IPF are burdened by an increased incidence of severe complicating events, apparently as lethal as the cancer itself.
[Show abstract][Hide abstract] ABSTRACT: The pulmonary fibrosis extent in systemic sclerosis (SSc) has a prognostic value. Chest Computed Tomography (CT) is the gold standard to detect an interstitial lung disease (ILD). Semi-quantitative scores and quantitative methods can estimate the ILD. The first ones have a considerable inter-intraobserver variability, while quantitative scores, based on distribution of lung attenuation parameters (also called CT indexes), can be obtained through expensive and not so user-friendly software. The aim of this work is to investigate whether a DICOM-viewer open-source software (OsiriX) can obtain CT indexes correlating with semi-quantitative scores. Sixty-three chest CTs of ILD-SSc patients were assessed with two semi-quantitative methods (visual extent and limited/extensive ILD grading) and then blindly processed with OsiriX to obtain the distribution parameters of lung attenuation (kurtosis, skewness and mean). Semiquantitative assessment and CT indexes were compared through the Spearman rank test and Mann-Whitney test. All CT indexes showed a statistically significant correlation of moderate degree with the visual extent semi-quantitative assessment (p-value less than 0.05). Skewness was the lung attenuation distribution parameter with the strongest correlation (r =-0.378, p-value = 0.0023). Moreover, CT indexes of patients with an extensive and limited disease were statistically different (p less than 0.01). CT indexes correlating with a radiological semi-quantitative ILD assessment can be obtained through OsiriX. CT indexes can be considered very helpful to discriminate patients with extensive and limited ILD.
Journal of biological regulators and homeostatic agents 07/2014; 28(3):507-13. · 2.41 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Mortality in pulmonary sarcoidosis is highly variable and a reliable prognostic algorithm for disease staging and for guiding management decisions is needed. The objective of this study is to derive and test a staging system for determining prognosis in pulmonary sarcoidosis.
We identified the prognostic value of high-resolution computed tomography (HRCT) patterns and pulmonary function tests, including the composite physiological index (CPI) in patients with pulmonary sarcoidosis. We integrated prognostic physiological and HRCT variables to form a clinical staging algorithm predictive of mortality in a test cohort. The staging system was externally validated in a separate cohort by the same methods of discrimination used in the primary analysis and tested for clinical applicability by four test observers.
The test cohort included 251 patients with pulmonary sarcoidosis in the study referred to the Sarcoidosis clinic at the Royal Brompton Hospital, UK, between Jan 1, 2000, and June 30, 2010. The CPI was the strongest predictor of mortality (HR 1·04, 95% CI 1·02-1·06, p<0·0001) in the test cohort. An optimal CPI threshold of 40 units was identified (HR 4·24, 2·84-6·33, p<0·0001). The CPI40, main pulmonary artery diameter to ascending aorta diameter ratio (MPAD/AAD), and an extent of fibrosis threshold of 20% were combined to form a staging algorithm. When assessed in the validation cohort (n=252), this staging system was strikingly more predictive of mortality than any individual variable alone (HR 5·89, 2·68-10·08, p<0·0001). The staging system was successfully applied to the test and validation cohorts combined, by two radiologists and two physicians.
A clear prognostic separation of patients with pulmonary sarcoidosis is provided by a simple staging system integrating the CPI and two HRCT variables.
National Institute of Health Research Respiratory Disease Biomedical Unit at the Royal Brompton and Harefield NHS Foundation Trust and Imperial College London.
The lancet. Respiratory medicine. 02/2014; 2(2):123-30.
[Show abstract][Hide abstract] ABSTRACT: Recent screening trial results indicate that low-dose computed tomography (LDCT) reduces lung cancer mortality in high-risk patients. However, high false-positive rates, costs, and potential harms highlight the need for complementary biomarkers. The diagnostic performance of a noninvasive plasma microRNA signature classifier (MSC) was retrospectively evaluated in samples prospectively collected from smokers within the randomized Multicenter Italian Lung Detection (MILD) trial.
Plasma samples from 939 participants, including 69 patients with lung cancer and 870 disease-free individuals (n = 652, LDCT arm; n = 287, observation arm) were analyzed by using a quantitative reverse transcriptase polymerase chain reaction-based assay for MSC. Diagnostic performance of MSC was evaluated in a blinded validation study that used prespecified risk groups.
The diagnostic performance of MSC for lung cancer detection was 87% for sensitivity and 81% for specificity across both arms, and 88% and 80%, respectively, in the LDCT arm. For all patients, MSC had a negative predictive value of 99% and 99.86% for detection and death as a result of disease, respectively. LDCT had sensitivity of 79% and specificity of 81% with a false-positive rate of 19.4%. Diagnostic performance of MSC was confirmed by time dependency analysis. Combination of both MSC and LDCT resulted in a five-fold reduction of LDCT false-positive rate to 3.7%. MSC risk groups were significantly associated with survival (χ1(2) = 49.53; P < .001).
This large validation study indicates that MSC has predictive, diagnostic, and prognostic value and could reduce the false-positive rate of LDCT, thus improving the efficacy of lung cancer screening.
Journal of Clinical Oncology 01/2014; · 17.88 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Sarcoidosis is a systemic granulomatous disorder of unknown aetiology with a wide spectrum of radiological appearances and almost invariably pulmonary involvement. Lung involvement accounts for most of the morbidity and much of the mortality associated with sarcoidosis. Imaging contributes significantly to the diagnosis and management of patients with sarcoidosis. In typical cases, chest radiography may be sufficient to establish the diagnosis with little margin of error and CT is not necessary. However, CT can play a critical role in several clinical settings: atypical clinical and/or radiographic findings; normal or near-normal chest radiograph but clinical suspicion of sarcoidosis; and detection of complications. Moreover, in many patients, CT findings are atypical and unfamiliar to most radiologists (e.g. sarcoidosis mimicking other lung diseases and vice versa), and in these cases histological confirmation of the diagnosis is recommended. CT is also useful in assessing disease extent and may help to discriminate between reversible and irreversible lung disease, thus providing critical prognostic information. This review concentrates on the more difficult imaging aspects of sarcoidosis, in particular differential diagnosis and disease complications. Key points •Sarcoidosis is characterized by a wide spectrum of radiological appearances.•In typical cases, imaging substantially contributes to the diagnosis of sarcoidosis.•CT plays a critical role in atypical and complicated cases.•CT may discriminate between reversible and irreversible lung disease.
[Show abstract][Hide abstract] ABSTRACT: Imaging makes a significant contribution to the diagnosis, prognosis and follow-up in sarcoidosis. Despite its increasing use, the role of computed tomography (CT) scanning in patients with known or suspected pulmonary sarcoidosis is still undefined. This review aims to compare the utility and limitations of chest radiograph and CT in patients with pulmonary sarcoidosis, with regards to the most critical clinical issues such as the diagnostic sensitivity, the differential diagnosis, and the prediction of the disease reversibility.
[Show abstract][Hide abstract] ABSTRACT: The diagnosis of pulmonary embolism (PE) is frequently considered in patients presenting to the emergency department or when hospitalized. Although early treatment is highly effective, PE is underdiagnosed and, therefore, the disease remains a major health problem. Since symptoms and signs are non specific and the consequences of anticoagulant treatment are considerable, objective tests to either establish or refute the diagnosis have become a standard of care. Diagnostic strategy should be based on clinical evaluation of the probability of PE. The accuracy of diagnostic tests for PE are high when the results are concordant with the clinical assessment. Additional testing is necessary when the test results are inconsistent with clinical probability. The present review article represents the consensus-based recommendations of the Interdisciplinary Association for Research in Lung Disease (AIMAR) multidisciplinary Task Force for diagnosis and treatment of PE. The aim of this review is to provide clinicians a practical diagnostic and therapeutic management approach using evidence from the literature.
Multidisciplinary respiratory medicine 12/2013; 8(1):75. · 0.05 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The multidisciplinary approach is ideal in the management of patients with lung cancer. Multidisciplinary evaluation strengthens the differential diagnosis of aspecific radiological findings, indeed. Notably, the differential diagnosis of early stage lung cancer is a current challenge of CT imaging because the earlier the detection, the lower the accuracy of radiological features. This is particularly true for the most common subtype of lung cancer, adenocarcinoma, because it shows various radiological features. Such variety is also reflected by the 2011 classification of lung cancer, that likely affected the diagnostic agreement between radiologist and clinician. This review discusses the common issues of lung cancer diagnosis by paired radiological-histologic interpretation of CT findings.
[Show abstract][Hide abstract] ABSTRACT: We read with interest the well-written study of Tane et al. about the usefulness of
320-multidetector row for preoperative three-dimensional (3D) pulmonary vasculature
assessment for candidates for pulmonary segmentectomies . Puzzlingly, quite
similar studies have been simultaneously published by other Japanese groups [2, 3].
We would like to add some considerations.
Importantly, this paper highlights the preoperative utility of identifying the intersegmental
vein to decide whether the segmentectomy is feasible or not. However, it
would be interesting to understand if the 3D software is now able to clearly depict
and differentiate pulmonary veins from arteries (e.g. by colour-coding these vessels
differently), thus facilitating the assessment of tumoural vein invasion. Furthermore, it
is unclear if the software can truly facilitate the identification of the intersegmental
pulmonary vein as compared to axial computed tomography images.
A second comment concerns the surgical technique: if a primary tumour is less
than 2 cm in diameter, is it really important to assess pulmonary vasculature precisely?
In fact, we think that it could be safe and sufficient (and probably easier) to identify
the lines of inflation and deflation to divide lung parenchyma while performing a
pulmonary segmentectomy. According to Schuchert and colleagues this approach
could decrease the risk of bleeding and prolonged postoperative air leaks . Maybe
a well designed randomized controlled trial would be able to clarify these open
Conflict of interest: none declared.
 Tane S, Ohno Y, Hokka D, Ogawa H, Tauchi S, Nishio W et al. The efficacy of
320-detector row computed tomography for the assessment of preoperative
980 S. Tane et al. / Interactive CardioVascular and Thoracic Surgery
pulmonary vasculature of candidates for pulmonary segmentectomy. Interact
CardioVasc Thorac Surg 2013;17:974–81.
 Iwano S, Yokoi K, Taniguchi T, Kawaguchi K, Fukui T, Naganawa S. Planning of
segmentectomy using three-dimensional computed tomography angiography
with a virtual safety margin: Technique and initial experience. Lung Cancer
 Saji H, Inoue T, Kato Y, Shimada Y, Hagiwara M, Kudo Y et al. Virtual segmentectomy
based on high-quality three-dimensional lung modelling from computed
tomography images. Interact CardioVasc Thorac Surg 2013;17:227–32.
 Schuchert MJ, Pettiford BL, Keeley S, D’Amato TA, Kilic A, Close J et al.
Anatomic segmentectomy in the treatment of stage I non-small cell lung
cancer. Ann Thorac Surg 2007;84:92–32.
Interactive Cardiovascular and Thoracic Surgery 12/2013; 17(6):980-1. · 1.11 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: PURPOSE : We aimed to compare the intra- and interoperator variability of lobar volumetry and emphysema scores obtained by semi-automated and manual segmentation techniques in lung emphysema patients. MATERIALS AND METHODS : In two sessions held three months apart, two operators performed lobar volumetry of unenhanced chest computed tomography examinations of 47 consecutive patients with chronic obstructive pulmonary disease and lung emphysema. Both operators used the manual and semi-automated segmentation techniques. The intra- and interoperator variability of the volumes and emphysema scores obtained by semi-automated segmentation was compared with the variability obtained by manual segmentation of the five pulmonary lobes. RESULTS : The intra- and interoperator variability of the lobar volumes decreased when using semi-automated lobe segmentation (coefficients of repeatability for the first operator: right upper lobe, 147 vs. 96.3; right middle lobe, 137.7 vs. 73.4; right lower lobe, 89.2 vs. 42.4; left upper lobe, 262.2 vs. 54.8; and left lower lobe, 260.5 vs. 56.5; coefficients of repeatability for the second operator: right upper lobe, 61.4 vs. 48.1; right middle lobe, 56 vs. 46.4; right lower lobe, 26.9 vs. 16.7; left upper lobe, 61.4 vs. 27; and left lower lobe, 63.6 vs. 27.5; coefficients of reproducibility in the interoperator analysis: right upper lobe, 191.3 vs. 102.9; right middle lobe, 219.8 vs. 126.5; right lower lobe, 122.6 vs. 90.1; left upper lobe, 166.9 vs. 68.7; and left lower lobe, 168.7 vs. 71.6). The coefficients of repeatability and reproducibility of emphysema scores also decreased when using semi-automated segmentation and had ranges that varied depending on the target lobe and selected threshold of emphysema. CONCLUSION : Semi-automated segmentation reduces the intra- and interoperator variability of lobar volumetry and provides a more objective tool than manual technique for quantifying lung volumes and severity of emphysema.
[Show abstract][Hide abstract] ABSTRACT: 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).
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.
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<0.001, 95% CI 1.57 to 4.77) and patients with discordant UIP had a better prognosis than concordant UIP but worse prognosis than fibrotic NSIP.
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.
[Show abstract][Hide abstract] ABSTRACT: Background: In 2002 the American Thoracic Society/European Respiratory Society (ATS/ERS) classification of idiopathic interstitial pneumonias (IIPs) defined seven specific entities, and provided standardized terminology and diagnostic criteria. In addition, the historical "gold standard" of histologic diagnosis was replaced by a multidisciplinary approach. Since 2002 many publications have provided new information about IIPs. Purpose: The objective of this statement is to update the 2002 ATS/ERS classification of IIPs. Methods: An international multidisciplinary panel was formed and developed key questions that were addressed through a review of the literature published between 2000 and 2011. Results: Substantial progress has been made in IIPs since the previous classification. Nonspecific interstitial pneumonia is now better defined. Respiratory bronchiolitis-interstitial lung disease is now commonly diagnosed without surgical biopsy. The clinical course of idiopathic pulmonary fibrosis and nonspecific interstitial pneumonia is recognized to be heterogeneous. Acute exacerbation of IIPs is now well defined. A substantial percentage of patients with IIP are difficult to classify, often due to mixed patterns of lung injury. A classification based on observed disease behavior is proposed for patients who are difficult to classify or for entities with heterogeneity in clinical course. A group of rare entities, including pleuroparenchymal fibroelastosis and rare histologic patterns, is introduced. The rapidly evolving field of molecular markers is reviewed with the intent of promoting additional investigations that may help in determining diagnosis, and potentially prognosis and treatment. Conclusions: This update is a supplement to the previous 2002 IIP classification document. It outlines advances in the past decade and potential areas for future investigation.
American Journal of Respiratory and Critical Care Medicine 09/2013; 188(6):733-48. · 11.04 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The imaging techniques in patients treated for lung cancer may be challenging to interpret. Radiologists are often asked to evaluate computed tomography (CT) scans after surgery, and this interpretation requires an understanding of both the timing and type of the surgical procedure. However, follow-up strategies are still not well defined. The assessment of tumor response to chemoradiotherapy relies on a tight integration of CT and clinical findings. Positron emission tomography-computed tomography (PET-CT) with fluorodeoxyglucose may help to exclude tumor recurrence when the sole CT scan is equivocal. More efforts are needed to validate the tools for volumetric tumor measurement in routine practice and to demonstrate their superiority compared to the Response Evaluation Criteria in Solid Tumors (RECIST). Familiarity with the assessment of lung cancer perfusion is also important because of the increasing use of cytostatic therapy. In this review, we outlined the imaging assessment of tumor recurrence after surgery and the role of CT, magnetic resonance imaging, and PET-CT in the follow-up after chemotherapy, radiotherapy, and radiofrequency ablation.