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Objectifs: La pneumopathie interstitielle (PI) représente la principale atteinte pulmonaire dans le syndrome de Gougerot-Sjögren primitif (SGSp). Un certain nombre de patients atteints de SGSp développent une forme fibrosante progressive de PI, mais il n'existe aucune donnée sur la prévalence de telles présentations.L'objectif de cette étude transversale monocentrique était d'explorer la prévalence des formes fibrosantes chez les patients atteints de SGSp présentant une PI. Méthodes: Tous les patients consécutifs remplissant les critères de classification du SGSp et présentant une PI existante ou nouvellement diagnostiquée ont été inclus dans l’étude. Le diagnostic de PI était toujours établi par TDM-HR et les différentes formes ont été déterminées sur la base des critères de classification en vigueur et réparties dans deux groupes selon qu'une forme fibrosante était détectée ou non. Résultats: Trente-quatre patients présentant un SGSp avec PI ont été inclus dans l’étude (3 hommes et 31 femmes, âge médian 69,5 ans, durée moyenne du SGSp 47,5 mois). Une forme fibrosante a été identifiée chez 52,9 % des patients (groupe 1) : pneumopathie interstitielle commune (PIC) chez 13 patients (38,2 %), pneumopathie interstitielle non spécifique (PINS) fibrosante chez 4 patients (11,8 %) et pneumopathie organisée (PO) fibrosante chez 1 patient (2,9 %). Le groupe 2 (16 patients, 47,1 %) comprenait la PINS chez 6 patients (17,6 %), la PO chez 4 patients (11,8 %), la PIL chez 2 patients (5,9 %) et la pneumopathie interstitielle inclassable chez 4 patients (11,8 %). Dans le groupe 1, les patients étaient plus jeunes et la durée du SGSp au moment du diagnostic de PI plus courte. Notamment, la PI a été diagnostiquée avant ou en même temps que le SGSp dans 83,3 % des cas, contre 62,5 % dans le groupe 2 de forme non fibrosante (p < 0,05). Conclusion: Nos données suggèrent une forte prévalence de ce phénotype clinique pulmonaire che

Objectives: This study aims to evaluate the diagnostic accuracy of the VECTOR software in patients with connective tissue diseases (CTDs), compared with the reference standard of high-resolution computed tomography (HRCT). Patients and methods: The study included 98 consecutive patients of CTD (24 males, 74 females; median age: 66 years; range, 24 to 85 years) with a recent HRCT. Patients were evaluated in a blindly manner by VECTOR and the results obtained by the algorithm were compared with the presence of interstitial lung disease (ILD) according to HRCT. Results: Interstitial lung disease was detected in 42.8% of subjects. VECTOR correctly classified 81/98 patients, with a diagnostic accuracy of 82.6%; sensitivity and specificity were 88.1% and 78.6%, respectively. Only 5/42 patients with ILD were not correctly classified by VECTOR, while false positive cases were 21.4%. No significant differences were observed according to the radiologic pattern of ILD. Conclusion: VECTOR showed high sensitivity, specificity and diagnostic accuracy, allowing selecting patients to be investigated with HRCT. The relatively high frequency rate of false positive results is acceptable if compared with the lack of effective screening methods for this complication of CTDs.

Background and objective: Recently the term “interstitial pneumonia with autoimmune features” (IPAF) has been proposed to identify patients with interstitial lung disease and autoimmune characteristics, not fulfilling the criteria for specific connective tissue diseases (CTD). Only few data are available about the clinical and serological features of IPAF patients, their survival and the possible evolution in a CTD. The aims of the study were to investigate the demographic and clinico-serologic features of patients with IPAF, their relationship to survival, and the possible evolution in a definite CTD. Patients and methods: Fifty-two patients were consecutively enrolled and prospectively followed for 45 ± 31.6 months. Data about disease onset, serological, clinical and therapeutic features, pulmonary function tests and high-resolution computed tomography were periodically repeated. The survival of patients with IPAF was compared with that of 104 patients with idiopathic pulmonary fibrosis (IPF). Results: The clinical domain for IPAF was satisfied in 44 patients, serological domain in 49 and the morphological domain in 29 patients. During the follow-up, a definite CTD was diagnosed in 7 patients, in particular Sjogren's syndrome in 4 patients, rheumatoid arthritis in 2, and polymyositis in the last. The estimated 5-year survival of IPAF patients 69.5 ± 7.8%, significantly higher than survival observed in IPF patients, and the baseline value of FVC and DLCO were the only factors associated to death. Conclusions: IPAF seems to a distinct entity, with a low tendency to evolve in a definite CTD. Nevertheless, further studies are needed to better define the clinical evolution and the outcome of IPAF.

In the article that appeared on Page 1621-1628, Vol 11, No 4 (April 2019) Issue of the Journal of Thoracic Disease (1), the given and family names of author “Fabrizio Pancaldi” was incorrectly published in the original. The author's name should be corrected as Fabrizio Pancaldi, instead of Pancaldi Fabrizio. The authors regret the error.

Background: Interstitial lung disease (ILD) is a severe systemic manifestation of rheumatoid arthritis (RA). High-resolution computed tomography (HRCT) represents the gold standard for the diagnosis of ILD, but its routine use for screening programs is not advisable because of both high cost and X-ray exposure. Velcro crackles at lung auscultation occur very early in the course of interstitial pneumonia, and their detection is an indication for HRCT. Recently, we developed an algorithm (VECTOR) to detect the presence of Velcro crackles in pulmonary sounds and showed good results in a small sample of RA patients. The aim of the present investigation was to validate the diagnostic accuracy of VECTOR in a larger population of RA patients, compared with that of the reference standard of HRCT, from a multicentre study. Methods: To avoid X-ray exposure, we enrolled 137 consecutive RA patients who had recently undergone HRCT. Lung sounds of all patients were recorded in 4 pulmonary fields bilaterally with a commercial electronic stethoscope (ES); subsequently, all HRCT images were blindly evaluated by a radiologist, and audio data were analysed by means of VECTOR. Results: Fifty-nine of 137 patients showed ILD (43.1%). VECTOR correctly classified 115/137 patients, showing a diagnostic accuracy of 83.9% and a sensitivity and specificity of 93.2 and 76.9%, respectively. Conclusions: VECTOR may represent the first validated tool for the screening of RA patients who are suspected for ILD and who should be directed to HRCT for the diagnosis. Moreover, early identification of RA-ILD could contribute to the design of prospective studies aimed at elucidating unclear aspects of the disease.

The circular economy can be promoted as a solution to support the sustainability market position of renewable energy systems. To design a circular and sustainable system, a structured approach is needed. The present study develops a methodology framework for sustainable circular system design (SCSD), aiming to assess thermal energy storage (TES) technologies from a sustainable perspective. To this end, a composite indicator, namely, environmental sustainability and circularity indicator (ESC) is provided. This indicator combines the environmental impacts of the TES system via the conduction of a life cycle assessment and its circulatory performance using the product-level material circularity indicator (MCI). The developed methodology is applied to a case study of high-temperature TES using molten salts as a part of a concentrated solar power plant. The SCSD embraces the analysis for the most relevant processes through proposing different ecological scenarios including, increasing the recycling rates (Modest Scenario), increasing the reuse rates (Medium Scenario), and a combination of both (Optimistic scenario). The circularity analysis showed that for the Modest, Medium and optimistic scenarios, the MCI moves from 20.6% for the current situation to 30.3%, 38.6%, and 46.4%, respectively. Accordingly, the optimistic scenario showed the most environmentally sustainable and circular scenario with ESC of 7.89%, whereas the Modest and Medium scenarios exhibited ESCs of 1.20% and 2.16%, respectively. A major obstacle for substantial improvement of the circulatory and ESC is the high share of unrecyclable molten salts in the system and therefore, any effort to improve the circulatory and the environmental benefits of this system can be reached by using more environmentally friendly alternative materials. The study concludes that the integration of reusing and recycling at the initial design should be sought in order to achieve a more environmentally sustainable and circula

Objectives: Interstitial lung disease (ILD) represents the main pulmonary involvement in primary Sjogren syndrome (pSS). A proportion of patients with pSS develop a progressive fibrosing form of ILD, but no data are available about the prevalence of these patterns in pSS patients. Aim of this monocentric, cross-sectional study was to investigate the prevalence of fibrosing patterns in pSS patients with ILD. Methods: All consecutive patients fulfilling classification criteria for pSS with a new or previous diagnosis of ILD were enrolled in the study. Diagnosis of ILD was always performed by mean of HRCT and specific patterns were identified according to current classification criteria and divided in two groups according to the detection of a fibrotic pattern. Results: Thirty-four pSS-ILD patients were enrolled in the study (males/females 3/31, median age 69.5 years, median pSS duration 47.5 months). Fibrotic pattern was detected in 52.9% of patients, namely: UIP (13 patients, 38.2%), fibrotic NSIP (4, 11.8%), fibrotic OP (1 2.9%) and group 2 (16 pts, 47.1%) including NSIP (6, 17.6%), OP (4, 11.8%), LIP (2, 5.9%) and unclassifiable (4, 11.8%). These patients were younger and with a shorter pSS duration at ILD diagnosis, in particular ILD diagnosis was prior or concurrent to pSS in 83.3% of cases compared to 62.5% in the group of nonfibrotic pattern (P < 0.05). Conclusion: Our data suggest a high prevalence of this pulmonary clinical phenotype in pSS-ILD patients. Since the course of progressive fibrosing pneumonia generally results in respiratory failure, this result could be worthy of further studies.

Acute exacerbation (AE) is a possible manifestation of interstitial lung diseases (ILD) associated to very high mortality. It’s defined as clinically significant respiratory deterioration with evidence of new widespread alveolar abnormalities on computed tomography scan. AE is better described in idiopathic pulmonary fibrosis (IPF) but also reported in ILD secondary to connective tissue diseases (CTD) and vasculitis. The main features and the real clinical impact of this severe complication in these patients are not well defined. Aim of our study was to prospectively investigate the incidence, clinical features and outcome of AE in a population of patients with ILD related to CTD and vasculitis. We consecutively enrolled all patients, with ILD secondary to rheumatic systemic diseases, referring to our multidisciplinary outpatient clinic for rare lung diseases. All patients were followed for at least 12 months (range, 12–36 months). At baseline, all patients underwent to a core set of laboratory investigations and periodically followed; data about demographic, disease onset, clinical, serological and therapeutic features were also recorded. AE occurred in 9/78 patients, with an incidence of 5.77/100 patients/year, and 5/9 patients died because of AE. The baseline value of DLCO was significantly associated to the risk of AE at Cox regression. In patients with ILD related to rheumatic systemic diseases AE can occur with an incidence similar to IPF. Rheumatologists should carefully consider this life-threatening complication as a possible natural course of all patients with ILD secondary to systemic rheumatic disease.

Objectives: To apply the Delphi exercise with iterative involvement of radiologists and pulmonologists with the aim of defining a structured reporting template for high-resolution computed tomography (HRCT) of patients with fibrosing lung disease (FLD). Methods: The writing committee selected the HRCT criteria—the Delphi items—for rating from both radiology panelists (RP) and pulmonology panelists (PP). The Delphi items were first rated by RPs as “essential”, “optional”, or “not relevant”. The items rated “essential” by < 80% of the RP were selected for the PP rating. The format of reporting was rated by both RP and PP. Results: A total of 42 RPs and 12 PPs participated to the survey. In both Delphi round 1 and 2, 10/27 (37.7%) items were rated “essential” by more than 80% of RP. The remaining 17/27 (63.3%) items were rated by the PP in round 3, with 2/17 items (11.7%) rated “essential” by the PP. PP proposed additional items for conclusion domain, which were rated by RPs in the fourth round. Poor consensus was observed for the format of reporting. Conclusions: This study provides a template for structured report of FLD that features essential items as agreed by expert thoracic radiologists and pulmonologists

The diagnosis of interstitial lung diseases in patients affected by rheumatoid arthritis is fundamental to improving their survival rate. In particular, the average survival time of patients affected by rheumatoid arthritis with pulmonary implications is approximately 3 years. The gold standard for confirming the diagnosis of this disease is computer tomography. However, it is very difficult to raise diagnosis suspicion because the symptoms of the disease are extremely common in elderly people. The detection of the so-called velcro crackle in lung sounds can effectively raise the suspicion of an interstitial disease and speed up diagnosis. However, this task largely relies on the experience of physicians and has not yet been standardized in clinical practice. The diagnosis of interstitial lung diseases based on thorax auscultation still represents an underexplored field in the study of rheumatoid arthritis. In this study, we investigate the problem of the automatic detection of velcro crackle in lung sounds. In practice, the patient is auscultated using a digital stethoscope and the lung sounds are saved to a file. The acquired digital data are then analysed using a suitably developed algorithm. In particular, the proposed solution relies on the empirical observation that the audio bandwidth associated with velcro crackle is larger than that associated with healthy breath sounds. Experimental results from a database of 70 patients affected by rheumatoid arthritis demonstrate that the developed tool can outperform specialized physicians in terms of diagnosing pulmonary disorders. The overall accuracy of the proposed solution is 90.0%, with negative and positive predictive values of 95.0% and 83.3%, respectively, whereas the reliability of physician diagnosis is in the range of 60−70%. The devised algorithm represents an enabling technology for a novel approach to the diagnosis of interstitial lung diseases in patients affected by rheumatoid arthritis.