The heterogeneity of lung involvement in vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic syndrome: a case of hypersensitivity pneumonitis-like pattern.

Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is a recently characterized disease associated with somatic mutations in the UBA1 gene, which cause dysregulation of ubiquitin-mediated processes. This case describes a 71-year-old male patient with VEXAS syndrome who presented with refractory lung inflammation with a pattern similar to computed tomography hypersensitivity pneumonitis, a novel finding in VEXAS syndrome. The presented clinical case highlights the protean involvement of the lung in VEXAS syndrome and emphasizes the importance of considering interstitial lung disease in the differential diagnosis.

Correlation Between Renal Cortical Stiffness and Histological Determinants by Point Shear-Wave Elastography in Patients With Kidney Transplantation.

INTRODUCTION: Renal allograft biopsy is the gold standard for the detection of histological lesions of chronic allograft dysfunction. The identification of a noninvasive routine test would be desirable. Elastosonography is used to assess tissue stiffness according to viscosity, and no data are available on the use of point quantification shear-wave elastography (ElastPQ) for the evaluation of renal chronic lesions. RESEARCH QUESTION: To evaluate the feasibility of ElastPQ to assess cortical allograft stiffness and to determine the correlation of clinical, biological, and pathological factors with the diagnostic accuracy of kidney stiffness values in patients with histological lesions. DESIGN: Forty-two patients underwent kidney transplant biopsy and 10 valid measurements of ElastPQ, blindly performed by 2 operators. The interobserver reproducibility was assessed according to intraclass correlation coefficient. The ElastPQ measurements and the clinical data were compared using the Spearman correlation analysis. RESULTS: 97.6% reliable measurements were obtained using ElastPQ, with an excellent interobserver agreement. The kidney stiffness was significantly higher in the patients with a time since transplantation >12 months and was correlated with chronic lesions (interstitial fibrosis, tubular atrophy transplant glomerulopathy, and mesangial matrix), with the interstitial fibrosis/tubular atrophy, score and with the sum of the scores of the chronic lesions. Mesangial matrix increase is the only independent determinant of kidney stiffness. DISCUSSION: ElastPQ is a noninvasive, reproducible, and sensitive diagnostic tool able to detect moderate/severe chronic lesions. Its routine use during follow-up can identify patients eligible for biopsy, which remains the gold standard exam for detecting chronic allograft dysfunction.

Screening for lung cancer using low-dose spiral CT: 10 years later, state of the art.

Ten years after the first papers on this subject, this editorial represents a brief review on lung cancer screening with low-dose spiral CT. The aim is to present the main theoretical and practical problems related to lung cancer screening, the historical background and results of observational studies and the main ongoing randomised controlled trials. In particular, the National Lung Screening Trial (NLST), which was interrupted early, is discussed. The opinion of the authors is that too many questions are still awaiting an answer.

Magnetic resonance imaging in the complications of kidney transplantation.

Kidney transplantation is currently the treatment of choice in most patients with end-stage chronic renal failure owing to the excellent results in terms of both graft and patient survival. However, surgical complications are still very frequent. Although urological (stricture, urinary fistulas, vesico-ureteral reflux) and lymphatic complications (lymphocoele) have a high incidence, they only rarely lead to graft loss. By contrast, vascular complications (stenosis, arterial and venous thrombosis, arterio-venous fistulas, pseudoaneurysms) are relatively rare, but potentially serious and may affect graft survival. Finally, medical complications such as acute tubular necrosis (ATN), rejection and de novo neoplasms may also arise in kidney transplantation. The purpose of this pictorial review is to illustrate the increasingly significant contribution of magnetic resonance angiography (MRA) in the management of complications of kidney transplantation, and emphasise how this method should now be considered a mandatory step in the diagnostic workup of selected cases. Moreover, the application and role in this setting of new magnetic resonance imaging (MRI) techniques, such as diffusion-weighted and blood oxygen level-dependent (BOLD) MRI, are also discussed.

Natural history of honeycombing: follow-up of patients with idiopathic pulmonary fibrosis treated with single-lung transplantation.

PURPOSE: Although honeycombing is one of the key features for the diagnosis of idiopathic pulmonary fibrosis (IPF), its origin and evolution are still poorly understood. The aim of our study was to analyse the natural history of honeycombing in patients treated with single-lung transplantation. MATERIALS AND METHODS: We considered seven patients who underwent single-lung transplantation; two of them (28.6%) were excluded from our analysis because they died in the posttransplantation period, whereas the remaining five (71.4%) were evaluated with computed tomography (CT) over 67.6±38.56 months. Each CT scan was assessed for disease extension and cyst size (visual score and size of target cysts); CT scans acquired after 2006 were also assessed for native lung volume. RESULTS: All patients showed disease progression (with a concurrent reduction in lung volume in two, 40%) and a progression of honeycombing, with increased number and size of cysts in four (80%). We observed dimensional changes in all target cysts (enlargement or reduction); three patients (60%) also had radiological evidence of complications, such as spontaneous rupture with pneumothorax and development of mycetomas within the cysts. CONCLUSIONS: Honeycombing is a dynamic process in which the overall trend is represented by a dimensional increase in cystic pattern; however, single cysts may have a different evolution (enlargement, reduction or complications). This behaviour could be explained by the variety of the pathogenetic processes underlying honeycombing, with cysts that may present abnormal communication with the airway, including the development of a check-valve mechanism.

Post-ARDS pulmonary fibrosis in patients with H1N1 pneumonia: role of follow-up CT.

PURPOSE: Our aim was to evaluate the evolution of 20 patients with H1N1 pneumonia, focusing our attention on patients with severe clinical and radiological findings who developed post-acute respiratory distress syndrome (post-ARDS) pulmonary fibrosis. MATERIALS AND METHODS: Twenty adult patients (nine women and 11 men; mean age 43.5 ± 16.4 years) with a diagnosis of H1N1 infection confirmed by pharyngeal swab came to our attention from September to November 2009 and were followed up until September 2010. All patients were hospitalised in consideration of the severity of clinical findings, and all underwent chest X-ray. Twelve of them underwent at least one computed tomography (CT) scan of the chest. RESULTS: In 75% of cases (15/20), there was complete resolution of the clinical and radiological findings. Twenty-five percent of patients (5/20) developed acute respiratory distress syndrome (ARDS), which progressed to predominantly peripheral pulmonary fibrosis in 10% (2/20; one died and one had late-onset pulmonary fibrosis, documented on day 68). Moreover, in one patient with a CT diagnosis of pulmonary fibrosis, we observed progressive regression of radiological findings over 4 months of follow-up. CONCLUSIONS: In patients with H1N1 pneumonia, post-ARDS pulmonary fibrosis is not a rare complication. Therefore, a CT scan should be performed in all patients with severe clinical findings. Our study demonstrated that in these patients, fibrosis could present a different spatial distribution and a different temporal trend, with delayed late onset; moreover, in one case, the signs of interstitial lung disease partially regressed over time. Therefore, CT should be considered not only in the diagnostic stage, but also during the follow-up.

Densitometric CT evaluation of acute and chronic thromboembolic filling defects of the pulmonary arteries before and after contrast injection.

PURPOSE: The aim of this study was to assess the baseline computed tomography (CT) attenuation of acute and chronic pulmonary thromboemboli, their contrast enhancement (CE), correlation with haematocrit (Ht) levels and the presence of hypertrophic bronchial arteries. MATERIALS AND METHODS: From January 2006 to October 2009, we measured the baseline and postcontrast attenuation values of acute pulmonary thrombi emboli on CT angiograms of 86 patients with acute pulmonary embolism (PE) and those of chronic thrombi in 29 patients with pulmonary hypertension of various origins. The attenuation of acute thrombi was correlated with Ht and CE of chronic thrombi with the presence of hypertrophic bronchial arteries. RESULTS: Acute emboli had a mean baseline attenuation of 54.9 Hounsfield units (HU) and showed no CE. The attenuation of acute thrombi was not dependent on Ht. Chronic thrombi had a mean baseline attenuation of 33.8 HU, and 54% of thrombi showed significant CE. In 57% of cases, a collateral circulation had developed. In 76.5% of cases, CE and hypertrophic bronchial arteries coexisted (p=0.026). Neither thrombotic CE nor bronchial artery hypertrophy predominated in any one of the diseases associated with chronic thrombosis. CONCLUSIONS: Before contrast administration, acute emboli coare prevalently hyperattenuating and therefore more conspicuous. Only chronic thrombi exhibit CE, and CE is significantly associated with the development of collateral circulation, which may be involved in the process of thrombotic recanalisation.

Follow-up in pulmonary sarcoidosis: comparison between HRCT and pulmonary function tests.

PURPOSE: The authors assessed the clinical usefulness of high-resolution computed tomography (HRCT) for monitoring sarcoidosis by comparing changes on HRCT with those on pulmonary function test (PFT) results over time. MATERIALS AND METHODS: The baseline and follow-up (after 13 months, range 15-63 months) HRCT scans of 14 consecutive patients with sarcoidosis were reviewed by a single observer. Each follow-up HRCT examination was assessed as stable, improved (when the extent of HRCT findings was reduced compared with baseline) and worsened (when the extent of HRCT findings was increased and/or when HRCT pattern had become fibrotic compared with baseline). Any increase or decrease in forced vital capacity (FVC)≥10% from baseline was considered significant. Changes on HRCT were then compared with those on FVC. RESULTS: During a median follow-up of 33 (range 15-63) months, HRCT findings worsened in 8/14 (58%) cases, improved in 3/14 (21%) and remained stable in 3/14 (21%). Agreement between changes on HRCT and FVC was moderate (κ=0.49). In 9/14 (64%) cases, HRCT changes were in line with those on FVC. In 4/5 discordant cases, the worsened HRCT findings were not mirrored by FVC changes. CONCLUSIONS: Despite the small size of our study population, our results suggest that HRCT may provide clinicians with additional information about the evolution of sarcoidosis.

Interstitial lung diseases in a lung cancer screening trial.

We assessed the prevalence of interstitial lung disease (ILD) in a cohort of smokers included in a lung cancer screening trial. Two observers independently reviewed, for the presence of findings consistent with ILD, the computed tomography (CT) examinations of 692 heavy smokers recruited by the Multicentric Italian Lung Detection (MILD) trial. Four CT patterns were considered: usual interstitial pneumonia (UIP), other chronic interstitial pneumonia (OCIP), respiratory bronchiolitis (RB) and indeterminate. Subsequently, the evolution of ILD in those subjects who had undergone a repeat CT examination after 3 yrs was assessed. The UIP pattern and the OCIP pattern were identified in two (0.3%) out of 692 and 26 (3.8%) out of 692 patients, respectively; 109 (15.7%) out of 692 patients showed CT abnormalities consistent with RB, while an indeterminate CT pattern was reported in 21 out of 692 (3%) patients. Age, male sex and current smoking status were factors associated with the presence of OCIP and UIP (combined) pattern, although the relationship did not attain statistical significance. A progression of the disease was observed in three (25%) out of 12 subjects with OCIP who underwent repeat CT after 3 yrs. Thin-section CT features of ILD, probably representing smoking-related ILD, are not uncommon in a lung cancer screening population and should not be overlooked.

Benefits of 3D technique in guiding percutaneous retroperitoneal biopsies.

PURPOSE: This study aimed to assess the usefulness and advantages of multiplanar reformations (MPR) during multidetector-row computed tomography (MDCT)-guided percutaneous fine-needle aspiration biopsy (FNAB) and core biopsy of retroperitoneal lesions that are difficult to access with the guidance of ultrasound and axial CT alone owing to overlying bony structures, large vessels or abdominal organs. MATERIALS AND METHODS: MDCT-guided retroperitoneal FNAB and core biopsy was performed on 14 patients with suspected retroperitoneal neoplasm. We used MPR images (sagittal and coronal) obtained with a six-detector-row MDCT scanner and 20-22 gauge Chiba needles. RESULTS: Using MDCT with 3D MPR allowed biological samples to be obtained in all cases (ten cytological and four histological) and diagnostic samples in 11/14 cases (78.5%). Histological samples were deemed adequate for diagnostic assessment in all cases and cytological samples in 7/10 cases (70%). CONCLUSIONS: MPR images allowed sampling of retroperitoneal lesions until now considered unreachable with the guidance of axial MDCT alone. Compared with the conventional procedure, the use of MPR images does not increase the procedure time.

Application of a new pulmonary artery obstruction score in the prognostic evaluation of acute pulmonary embolism: comparison with clinical and haemodynamic parameters.

PURPOSE: Evaluation of computed tomography (CT) pulmonary angiography parameters revealing pulmonary embolism (PE) severity with particular attention to pulmonary obstruction indexes. Comparison with clinical and hemodynamic data and determination of predictive role in the development of chronic pulmonary heart disease. MATERIALS AND METHODS: This retrospective study analyzes 45 not consecutive patients from November 2007 to December 2008 with CT angiography diagnosis of acute PE. Included in the study are patients at the first documented episode of acute PE, with 6 month follow-up. Patients with severe pre-existent cardiopulmonary pathology or neoplastic diseases were excluded from the study. CT angiography evaluated right ventricular (RV)/left ventricular (LV) ratio, obstruction index according to Qanadli and Total Clot Burden (Ghanima score). PE indexes were compared with Troponin I measurement and echocardiography result; at last hospitalization and intensive care time were reported. RESULTS: A significant association was found between Ghanima and Qanadli score: the two indexes are equivalent in quantification of pulmonary arterial obstruction (p<0.001). Among others CT parameters, the new Ghanima score evidenced the best accuracy to detect patients evolving to chronic pulmonary heart disease (76%). This value is higher than that of echocardiography (71%). Troponins showed highest accuracy (82%). CONCLUSIONS: Ghanima score can be used in emergency CT angiography diagnosis as prognostic marker for a quickly risk stratification of pulmonary heart disease or death in patients with acute PE. This approach allows to obtain, with just one test, both the diagnosis and a rather accurate acute PE risk stratification.

Critical issues in radiology requests and reports.

PURPOSE: This study evaluated the appropriateness and accuracy of 500 radiology requests and their matched reports in order to identify recurring errors in both areas. MATERIALS AND METHODS: A randomly chosen sample consisting of 167 computed tomography (CT), 166 ultrasonography (US) and 167 radiographic examinations were collected and analysed according to national referral guidelines and to the principles of justification and optimisation (Law no. 187/2000). RESULTS: We identified a high rate of inappropriate requests (27.6%) and requests lacking a clinical question (22%). There was good precision in the anamnestic data (80.6%) and in the formulation of the diagnostic question (76.8%). Almost all requests were handwritten, and 12.5% lacked the referring physician's stamp and/or signature. No report mentioned the clinical information received or the equipment used. The use of contrast medium was always reported. Conclusions were reported in 9.8% of these reports. When further investigation would have been necessary, the radiologist omitted to report this in 60% of cases. CONCLUSIONS: Some important weaknesses emerged, especially regarding requests for radiological examinations (22% lacked the clinical question, 27.6% were inappropriate), potentially limiting the effectiveness of the diagnostic process and leading to negative effects on the correct risk management process. There emerges a need for better collaboration between clinicians and radiologists.

High-resolution CT in smoking-related interstitial lung diseases.

In addition to chronic obstructive pulmonary disease (COPD) and bronchogenic carcinoma, smoking can also cause interstitial lung diseases (ILDs) such as respiratory bronchiolitis (RB), RB with ILD (RB-ILD), desquamative interstitial pneumonia (DIP), Langerhans cell granulomatosis (LCG) and idiopathic pulmonary fibrosis-usual interstitial pneumonia (IPF-UIP). However, smoking seems to have a protective effect against hypersensitivity pneumonitis (HP), sarcoidosis and organising pneumonia (OP). High-resolution computed tomography (HRCT) has a pivotal role in the differential diagnosis. RB is extremely frequent in smokers, and is considered a marker for smoking exposure. It has no clinical relevance in itself since most patients with RB are asymptomatic. It is frequent to observe the association of RB with other smoking-related diseases, such as LCG or pulmonary neoplasms. In RB-ILD, HRCT features are more conspicuous and diffuse than in RB, but there is no definite cut-off between the two entities and any distinction can only be made by integrating imaging and clinical data. RB, RB-ILD and DIP may represent different degrees of the same pathological process, consisting in a bronchiolar and alveolar inflammatory reaction to smoking. Smoking is also a well-known risk factor for pulmonary fibrosis. Multidisciplinary discussion and follow-up can generally solve even the most difficult cases.

Lung function predicts lung cancer risk in smokers: a tool for targeting screening programmes.

The relationship between smoking, lung cancer and airflow obstruction is recognised but it is unclear whether the presence of minimal lung function damage constitutes an independent risk factor for the development of lung cancer. In order to identify those individuals at higher risk of lung cancer on the basis of functional impairment, we evaluated baseline pulmonary function tests of 3,806 heavy smokers undergoing annual chest computed tomography screening, and compared the forced expiratory volume in 1 s (FEV(1)) % predicted of 57 lung cancer cases and that of 3,749 subjects without cancer. We obtained odds ratios (ORs) of lung cancer and the corresponding 95% confidence intervals (CIs) using unconditional logistic regression, adjusting for age, sex, study and smoking variables. Compared with subjects with FEV(1) >or=90% pred, the OR of lung cancer was 2.45 (95% CI 1.39-4.33) for subjects with FEV(1) <90% pred and 2.90 (95% CI 1.34-6.27) for subjects with FEV(1) <70% pred. These data show that even a relatively small reduction in FEV(1) % pred is a significant predictor of increased lung cancer risk. Test screening for lung cancer using airflow obstruction with FEV(1) <90% is a strategy worth future consideration.

High-resolution computed tomography in the diagnosis and follow-up of idiopathic pulmonary fibrosis.

Idiopathic pulmonary fibrosis (IPF) is the most common interstitial lung disease and is associated with a fatal prognosis. Familiarity with the typical appearances of IPF on high-resolution computed tomography (HRCT) is important, as in the appropriate clinical setting, it is often sufficient for establishing a confident diagnosis of IPF without the need for surgical biopsy. Moreover, HRCT can provide important prognostic information in IPF. This is noteworthy, as the course of IPF is variable, and many patients develop complications leading to respiratory failure and death. The purpose of this paper is to review the progress made towards a better understanding of the HRCT patterns of IPF.

Pancreatic and extrapancreatic lesions in patients with intraductal papillary mucinous neoplasms of the pancreas: a single-centre experience.

PURPOSE: This study was performed to describe the possible presence of extrapancreatic neoplasms in patients with intraductal papillary mucinous neoplasm (IPMN) and to evaluate whether the extrapancreatic tumours were synchronous or metachronous to IPMNs. MATERIALS AND METHODS: One hundred and forty-two patients (56 men and 86 women; mean age 69.5 years, range 37-98) with IPMN diagnosed using the Sendai criteria were enrolled. Six patients (4.2%) had type I, 103 (72.5%) type II and 33 (23.2%) type III IPMN. All patients were studied using the following imaging techniques: ultrasonography (US), multidetector computed tomography (MDCT) and magnetic resonance cholangiopancreatography (MRCP). RESULTS: Pancreatic IPMN was localised in the head in 43 patients (30.3%), in the body in 13 (9.2%), in the tail in ten (7.0%), in the head-body in 17 (12.0%), in the body-tail in 15 (10.6%) diffuse throughout the gland in 44 (31.0%). The mean size of the cystic lesions was [(mean+/-standard deviation (SD)] 1.9+/-1.9 cm (range 0.5-8.0 cm). Twenty patients (14.1%) had associated pancreatic or extrapancreatic diseases. In evaluating the distribution of pancreatic or extrapancreatic diseases according to IPMN type, we found that this was not significantly different among types I, II and III (p=0.776). CONCLUSIONS: The majority of pancreatic and extrapancreatic cancers occur before the diagnosis of IPMNs is made and is not related to the type of IPMN.

Three-dimensional analysis of pulmonary nodules: variability of semiautomated volume measurements between different versions of the same software.

PURPOSE: This study was done to evaluate the variability of semiautomated volume measurements of solid pulmonary nodules between two different versions of the same volumetric software. MATERIALS AND METHODS: The volumes of 100 solid intraparenchymal nodules (mean volume 88.10 mm(3); range 7.36-595.25 mm(3)) studied with the same multidetector computed tomography (MDCT) protocol were determined using two different versions of the same volumetric software (LungCARE 2006G and LungCARE 2007S). The 2006G version is based on a single-segmentation algorithm, whereas the newer version features two algorithms: SmallSizeNodule and AllSizeNodule. The results obtained with the 2006G version were compared with those of the 2007S version with the SmallSizeNodule algorithm, as recommended by the user manual. In addition, we compared the volumetric measurements obtained by the two different algorithms of the 2007S version. RESULTS: The 2006G version and the 2007S version with the SmallSizeNodule algorithm agreed in only two of 100 cases and showed a mean variability of 1.66% (range 0%-8.78%). A more significant volumetric discrepancy was observed between the two different algorithms of the 2007S version, with the AllSizeNodule algorithm providing on average larger volumes (mean variability 71.08%; range 6.02%-218.80%) than SmallSizeNodule. Volume discrepancies were more pronounced in the subgroups of smaller nodules in all comparisons. CONCLUSIONS: There is variability also in the results provided by different versions of the same volumetric software, and this may affect the calculation of the nodule-doubling time. Computer-aided assessment of the growth of lung nodules should always be performed using the same version of volumetric software and the same segmentation algorithm.