Detection Of Interstitial Lung Disease Research Articles

Comparison between Conventional Radiography and High Resolution Computed Tomography in Interstitial Disease of Lungs

Background : Interstitial lung diseases are a diverse group of over 200 disease entities in which the primary site of injury is the lung interstitium. These diseases vary widely in their etiology, clinicoradiologic presentation, histopathological features and clinical course. HRCT has its limitations in the detection of interstitial lung diseases, its sensitivity is not 100% and the limitations of sensitivity are not well established. This study attempted to compare between conventional radiography and HRCT in the diagnosis of interstitial lung diseases. Subjects and Methods: All the patients were subjected to both conventional radiography and HRCT examinations and the images were viewed and analyzed. The two modalities were compared with regards to their ability to detect findings like nodular opacities, reticular opacities, septal thickening, honeycombing, lymphadenopathy etc. Results: The study has gender distribution of 60% males and 40% females. The main result of the study was that higher number of samples with findings were detected by HRCT than conventional radiography. In the detection of nodular opacity and septal thickening, the p value was less than 0.05 meaning the difference was statistically significant. Of the 30 patients, 2 had normal chest radiograms while HRCT was able to detect reticular opacities in these patients. The most common abnormality seen in both chest radiograms and HRCT was reticular opacities. The distinction between air space nodules and interstitial nodules was also much better appreciated on HRCT than on conventional radiography. In the detection of honeycombing, HRCT was much more specific being able to detect it in cases when the chest radiogram showed only reticular opacities. Even in the detection of other findings like air trapping and lymphadenopathy, HRCT scored over conventional radiography. Conclusion: HRCT therefore seems to be the investigation of choice in evaluating patients of interstitial lung disease.

Diagnostic Accuracy of Multidetector CT in Detection of Early Interstitial Lung Disease With Its Role in Characterization.

BackgroundMultidetector CT (MDCT) has emerged as a useful option for early diagnosis of interstitial lung disease (ILD) with adequate accuracy.MethodsA total of 80 patients with restricted pulmonary functions and clinical suspicion of ILD were enrolled in the study. MDCT evaluation was done using Siemens Somatom Force 384 slice multidetector computer tomography machine. Pattern analysis for reticular opacities, nodules and lung opacities was done to reach at a diagnosis. Final diagnosis was based on correlation of radiological and clinicopathological findings. Diagnostic efficacy of MDCT was evaluated in terms of sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV) and accuracy for detection of ILD.ResultsMean age of patients was 58 ± 8.75 years. Majority were females (51.3%). History of chronic obstructive pulmonary disease (COPD), tuberculosis and bronchial asthma was revealed in 31 (38.8%), 26 (32.5%) and 16 (20%) patients, respectively. There were 30 (37.5%) patients having no history of respiratory diseases. MDCT diagnosed ILD in 45 (56.3%) cases. On final diagnosis, ILD was diagnosed in 35 (43.8%) cases (15 usual interstitial pneumonia [UIP], 9 cryptogenic organizing pneumonia [COP], 8 nonspecific interstitial pneumonia [NSIP] and 3 respiratory bronchiolitis associated interstitial lung disease [RBILD]). The sensitivity, specificity, positive predictive value, negative predictive value and accuracy of MDCT in detection of ILD was 91.4%, 71.1%, 71.1%, 91.4% and 80%, respectively.ConclusionMDCT as a single modality had a high sensitivity for detection of ILD and could be recommended as first line diagnostic imaging technique.

EP29 An evaluation of the use of baseline pulmonary function tests in the detection of interstitial lung disease in patients newly diagnosed with rheumatoid arthritis

Abstract Background Interstitial lung disease (ILD) is a serious extra-articular manifestation of rheumatoid arthritis (RA). Risk factors include smoking, the presence of rheumatoid factor (RF) and anti-cyclic citrullinated peptide antibodies (CCP). Pulmonary function tests (PFT) show reduced carbon monoxide diffusion capacity (DLCO) early and reduced forced vital capacity (FVC) later in disease. HRCT is the gold standard diagnostic test while chest X-ray (CXR) has low sensitivity. PFT are routinely performed in the majority of RA patients at baseline at our tertiary centre. The aim of this study was to evaluate the frequency of abnormal PFT, specificity for ILD and influence on subsequent decision-making in patients newly diagnosed with RA. Methods A retrospective analysis was undertaken of patients with a new diagnosis of RA between January 2016 and December 2017. Patients meeting the ACR (2010) criteria for RA, with baseline PFT data available were included. Clinic letters and the hospital electronic records were used to obtain the data. Results 139 patients were included in the data analysis (Table 1). 23 patients had DLCO <70% predicted, while 7 patients had an FVC <80% predicted. Patients with abnormal PFT were more likely to be older, female, seropositive and to have smoked. Of the patients with DLCO <70%, CXR was abnormal in 6 patients with changes suggesting ILD in 2 patients. 13 patients had HRCT and 7/13 patients had evidence of ILD and 6/13 patients had significant emphysema on CXR or HRCT. 1 patient with DLCO of 82% had changes of ILD on a CT scan organised for another reason. Methotrexate was commenced in 19/23 patients with DLCO<70% and discontinued in 2 patients for respiratory reasons. Conclusion This evaluation suggests baseline PFT are more sensitive than baseline CXR in detecting ILD but that a DLCO <70% is not specific for this diagnosis. The abnormal PFT lead to HRCT being requested in 13/24 patients, of whom 7 had ILD which had not been identified by CXR in 5 patients. Baseline PFT are also useful as a reference point in patients who go on to develop respiratory symptoms at a later point in their illness. Disclosures A. Haque None. R. Kilding None. R. Smith None. S. Khalid None. R. Sandler None. M. Cox None. T. Hendry None. A. Flores-martin None. K. Lindop None. J. Maxwell None.

Increased serum soluble programmed death ligand 1(sPD-L1) is associated with the presence of interstitial lung disease in rheumatoid arthritis: A monocentric cross-sectional study.

This paper is to examine the relationship between serum soluble programmed death ligand 1(sPD-L1) levels and the development of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA). Serum sPD-L1 were measured by enzyme-linked immunosorbent assay. sPD-L1 levels in RA with ILD, RA without ILD and healthy controls were compared. Associations between ILD and various markers including sPD-L1 and confounding factors were investigated by logistic regression analysis. Diagnostic values of sPD-L1 for the presence of ILD were investigated using receiver operating characteristics curve analysis. Serum sPD-L1 levels were higher in RA patients with ILD than RA patients without ILD and healthy controls (23.7±9.8 vs. 18.0±7.7pg/mL, P=0.01 and 23.7±9.8 vs. 2.9±1.5pg/mL, P<0.0001). sPD-L1 levels were positively correlated with RF titer (r=0.245, P=0.03), CRP (r=0.265,P=0.01), HRCT score (r=0.265, P=0.04) and Ferritin (r=0.442, P=0.01), but negatively associated with FVC% (r=-0.359, P=0.01) and DLCO% (r=-0.399, P=0.008). sPD-L1 and anti-CCP antibody status were independently associated with the presence of ILD during multivariate logistic regression analysis. Sensitivity and specificity of sPD-L1 levels for the detection of ILD in RA patients were 51.7% and 79.3%, respectively (area under the curve=0.661). Serum sPD-L1 levels were increased in RA patients with ILD. Increased sPD-L1 levels were associated with the presence of ILD.

Deep learning for screening of interstitial lung disease patterns in high-resolution CT images

To develop a screening tool for the detection of interstitial lung disease (ILD) patterns using a deep-learning method. A fully convolutional network was used for semantic segmentation of several ILD patterns. Improved segmentation of ILD patterns was achieved using multi-scale feature extraction. Dilated convolution was used to maintain the resolution of feature maps and to enlarge the receptive field. The proposed method was evaluated on a publicly available ILD database (MedGIFT) and a private clinical research database. Several metrics, such as success rate, sensitivity, and false positives per section were used for quantitative evaluation of the proposed method. Sections with fibrosis and emphysema were detected with a similar success rate and sensitivity for both databases but the performance of detection was lower for consolidation compared to fibrosis and emphysema. Automatic identification of ILD patterns in a high-resolution computed tomography (CT) image was implemented using a deep-learning framework. Creation of a pre-trained model with natural images and subsequent transfer learning using a particular database gives acceptable results.

Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention.

Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.

Subclinical Interstitial Lung Disease in Patients with Systemic Sclerosis. A Pilot Study on the Role of Ultrasound.

Interstitial lung disease (ILD) is a common comorbidity present in patients with systemic sclerosis (SSc). Employment of high-resolution computed tomography (HRCT) is very limited and lung ultrasound (LUS) can be an alternative tool for the early evaluation of ILD. To determine the validity of LUS in the early detection of ILD in patients with SSc. Sixty-eight patients with SSc ≥18 years without respiratory symptoms were included. A rheumatologist rated the subclinical respiratory condition, another rheumatologist blinded to the clinical assessment performed the LUS. To determine validity HRCT was performed as well. Prevalence of ILD in SSc patients was 41.2% in contrast to the 4.8% healthy controls (P=.0001). Variables associated with LUS and HRCT findings were anti-centromere antibodies (P=.005) and the Rodnan skin score (P=.004). A positive correlation was present between the findings of HRCT and LUS (P=.001). Sensitivity and specificity were 91.2% and 88.6% respectively. Good reliability in the LUS findings was found between observers (k=.72). By proving to be a valid, trustworthy and feasible alternative tool, we consider that LUS can be implemented for the early detection of ILD in SSc.

Evaluation and management of Idiopathic Pulmonary Fibrosis.

Idiopathic Pulmonary Fibrosis (IPF) is a most common progressive interstitial lung disease (ILD) of unknown etiology, although majority of patients are elderly male smokers. The main pathogenesis is aberrant recovery of epithelial injury and collagen deposition. Fibrotic nonspecific interstitial pneumonia, connective tissue disease (CTD) especially rheumatoid arthritis (RA) associated ILD, and chronic hypersensitivity pneumonia(CHP) are important differential diagnosis. Main symptoms are non-productive cough and progressive exertional dyspnea. Crucial physical findings are scalene muscle hypertrophy, bibasilar fine crackles, and finger clubbing. The serum markers such as lactate dehydrogenase (LDH) and Krebs von den Lungen-6 (KL-6) are sensitive for ILD detection and activity. Both pulmonary function test (PFT) and the 6-minute walk test (6MWT) are useful tool for evaluation of disease progression of IPF. Serial changes of forced vital capacity (FVC) and 6MWT distance predict mortality in IPF effectively. Recently published international IPF guidelines highlight the importance of chest high resolution computed tomography (HRCT) findings such as honeycombing, traction bronchiectasis (TBE), and sub-pleural reticular opacity. IPF is chronic and progressive; therefore, tracking disease behavior is crucial. Unifying clinical, physiological, and imaging information over time is useful. With regard to its management, two anti-fibrotic drugs such as pirfenidone and nintedanib have been available. These drugs can slow the decline of FVC and prevent acute exacerbation (AE). In this review, I outline the clinical characteristics of IPF, physiological, imaging, pathological findings and review diagnosis process and management.

Clinical case of community-aquaired pneumonia on the background of previously undiagnosed occupational pulmonary fibrosis. Is occupational history important?

Interstitial lung disease is a systemic concept, which is understood as a group of progressive diseases, characterized by non-infectious inflammation in the wall of the alveoli and interstitial lung tissue and may result in pulmonary fibrosis. The publication deals with the clinical case of community-acquired pneumonia on the background of previously undiagnosed occupational pulmonary fibrosis. The complaints, anamnesis, clinical and instrumental data have been analyzed, special attention is paid to the professional history, which indicates long-term contact with toxic substances and negative factors of the industrial process. The data of pathomorphological research are given. Timely prevention and screening of workers involved in heavy work and work with harmful or hazardous working conditions, even after the cessation of work in a production environment, may contribute to early detection of interstitial lung disease and contribute to a positive prognosis for acute respiratory diseases occurring on its background.

Interleukin-18 is associated with the presence of interstitial lung disease in rheumatoid arthritis: a cross-sectional study

Objective: Serum interleukin-18 (IL-18) levels are increased in patients with interstitial lung disease (ILD). In addition, IL-18 levels are increased in patients with rheumatoid arthritis (RA) and are associated with arthritis activity. We determined whether increased IL-18 levels are associated with ILD in RA.Method: RA patients were enrolled using an RA cohort database. Plasma IL-18 levels were measured by enzyme-linked immunosorbent assay. ILD was determined by a pulmonologist and a radiologist based on chest radiography and computed tomography findings. IL-18 levels for RA with ILD and RA without ILD were compared. Associations between ILD and various markers including IL-18 and confounding factors (e.g. smoking history) were investigated by logistic regression analysis. Diagnostic values of IL-18 for the presence of ILD were investigated using receiver operating characteristics curve analysis.Results: ILD was complicated in 8.2% (n = 26) of the study population (N = 312). Plasma IL-18 levels were higher for RA patients with ILD than for RA patients without ILD (721.0 ± 481.4 vs 436.8 ± 438.9 pg/mL, p < 0.001). IL-18, Krebs von den Lungen-6, and anti-cyclic citrullinated peptide antibody titre and glucocorticoid doses were independently associated with the presence of ILD during multivariate logistic regression analysis. Sensitivity and specificity of IL-18 levels for the detection of ILD in RA patients were 65.3% and 76.3%, respectively (area under the curve = 0.73).Conclusion: Plasma IL-18 levels were higher for RA patients with ILD than for those without ILD. Increased IL-18 levels were associated with the presence of ILD.

Matrix Metalloproteinase 7 in Rheumatoid Arthritis Patients with Interstitial Lung Disease

The study aim: The purpose of this study is to measure the matrix metalloproteinase 7 (MMP7) in rheumatoid arthritis (RA) patients with interstitial lung disease (ILD) and to assess for any correlation with RA-disease activity. Research design: A cross sectional study. Sample: a purposive sample included 40 rheumatoid arthritis patients from Ain Shams University Hospitals Inpatient Department of Rheumatology and Outpatient Clinic. The results revealed that serum (S) MMP7 was significantly higher among RA patients with interstitial lung disease than patients without. Conclusion: The study documented that S.MMP7 may be used as a screening test for detection of interstitial lung disease in patients with rheumatoid arthritis. The study recommended: Measurement of serum MMP7 level to RA patients may be used as screening test for detection of ILD

Diagnostic Utility of 3T Lung MRI in Children with Interstitial Lung Disease: A Prospective Pilot Study

The objective of this study was to assess the diagnostic utility of 3-tesla (3T) magnetic resonance imaging (MRI) of lungs in the detection of interstitial lung disease (ILD) in pediatric patients. Twelve children (mean: 8.5 years, range: 4-12 years) with ILD were consecutively enrolled in this prospective study. HRCT and 3T lung MRI were performed in all patients within 2 days of each other. The sensitivity, the specificity, the positive predictive value, and the negative predictive value of detecting lung abnormalities related to ILD with 3T lung MRI were calculated, with high-resolution computed tomography (HRCT) as a standard of reference. Agreement between HRCT and 3T lung MRI, as well as between two reviewers, was calculated with the kappa coefficient. 3T lung MRI had low sensitivity (66.67%) and high specificity (97.33%) in the detection of abnormalities related to ILD when compared to HRCT in children. Although 3T lung MRI performed well in the detection of consolidation, parenchymal bands and fissural thickening with a sensitivity of 100%, the sensitivity of 3T lung MRI in the detection of septal thickening, ground-glass opacity, nodules, and cysts was relatively low (50.0%, 50.0%, 66.67%, and 25.0%, respectively). Substantial agreement was seen between HRCT and 3T lung MRI (k = 0.7), whereas perfect agreement was seen between two reviewers in detecting abnormalities related to pediatric ILD (k = 0.9-1.0). In comparison to HRCT, 3T lung MRI with routinely available MRI protocols and sequences can also well detect abnormalities such as consolidation, parenchymal bands, and fissural thickening in children with ILD. However, evaluation of septal thickening, ground-glass opacity, nodules, and cysts is limited with 3T lung MRI.

Computer-aided detection of interstitial lung diseases: A texture approach

We have developed the flexible scheme for computer-aided detection (CAD) of interstitial lung diseases on chest radiographs. These schemes enable us to perform diagnostics in the broad circumstances of pneumonia and other interstitial lung diseases. It is applied in the case of children pneumonia when conditions are difficult to standardize. In the adults' case the schemes of CAD are more adaptive, as there are more characteristic interstitial lung tissue's changes to all kinds of pathological conditions. Even in the norm of drawing there are more visible and more highlighted features, leading to better results. The CAD scheme works as follows. For the first of all, we are using adopted algorithms of active contours to select the area of lungs, and then to divide this area into subareas - regions of interest (40 different ROI). Then ROIs were subjected to the 2-dimensional Daubechies wavelet transform, and only main transformation was used. For every transformation 12 texture measures were calculated. Principal component analysis (PCA) was used to extract 2 main components for each ROI, and these components were compared to predictive component region.

Being aware of potentially fatal interstitial lung disease in patients with head and neck cancer treated with concurrent cetuximab and radiotherapy

AbstractCetuximab-induced interstitial lung disease (ILD), especially for tumors in the head and neck area, has rarely been reported. The present case may serve as an important reference to alert clinicians who use this chemotherapeutic agent. We report a case of acute ILD in a 66-year-old male with double cancers (oral floor cancer T4aN0M0 and supraglottic cancer T2N0M0), who received concurrent cetuximab and radiation therapy. He had severe dyspnea on day 51 along with worsened bilateral, progressive infiltration in chest X-ray, elevated C-reactive protein, Krebs von den Lungen 6 (KL-6), Surfactant Protein D (SP-D) and Surfactant Protein A (SP-A), and was diagnosed with ILD. He was treated with High-dose methylprednisone (1 mg/kg) soon after the diagnosis was made. He recovered remarkably and was discharged at day 100. For early detection of ILD, we suggest weekly chest X-rays for cetuximab-treated patients. In suspicious cases, High-resolution computed tomography scan and measurements of KL-6, SP-A, an...

Integrated Use of Lung Ultrasound and Chest X-Ray in the Detection of Interstitial Lung Disease

Background: Current data have shown that lung ultrasound (LUS) may be useful in the detection of interstitial lung disease (ILD) by the evaluation of B-lines, the sonographic marker of pulmonary interstitial syndrome. Nevertheless, no prospective study has compared LUS to chest X-ray (CXR) for ILD assessment, and there is no general agreement on the specific echographic diagnostic criteria for defining ILD. Objectives: The aims of this study were (1) to compare the accuracy of LUS and CXR in the detection of ILD using high-resolution CT (HRCT) as the gold standard and (2) to compare the accuracy of different echographic diagnostic criteria for ILD diagnosis. Methods: LUS was performed on 104 patients undergoing HRCT for suspected ILD. In 49 patients, a CXR scan performed within 3 months of HRCT was analyzed. ILD was defined as the presence of ≥5 B-lines in ≥3 chest areas. A total B-line score (TBLS) was also calculated, as in previous studies. The observers evaluating LUS and CXR were blinded to the HRCT results and clinical data. Results: On HRCT, ILD was assessed in 50 patients. CXR was specific (91%; 95% CI 80-100) but not sensitive (48%; 95% CI 28-67). Conversely, LUS showed high sensitivity (92%; 95% CI 84-99) and low specificity (79%; 95% CI 69-90). Using a TBLS, sensitivity did not change, while specificity decreased. Conclusions: LUS could be a sensitive tool for ILD detection. CXR and LUS have different but complementary features, and their combined use could reduce the need for HRCT. The use of different diagnostic criteria for defining ILD does not affect sensitivity but influences specificity.

Interstitial lung disease in systemic sclerosis: progress in screening and early diagnosis.

Interstitial lung disease (ILD) is the major determinant of morbidity and mortality in systemic sclerosis (SSc). In highly selected SSc patients, it was recently shown that stem cell therapy early in the disease course improved survival and reduced the extent of ILD, providing a rationale for early ILD detection strategies in this disease. Here, we review recent progress on ILD screening and early diagnosis in SSc. Two studies showed that over 60% of unselected SSc cases with ILD by high-resolution computer tomography (HRCT) had normal range pulmonary function tests (PFTs); indicating poor performance of PFTs for ILD screening purposes. Serial, paired HRCT and PFT analyses indicated that screening by HRCT at baseline predicted risk for lung fibrosis development, progression rate of fibrosis and PFT decline. Analyses of circulating biomarkers, like CCL18, and nonradiating lung imaging modalities, like ultrasound and MRI, showed promise as tools for early ILD detection; but further work is needed. Prospective cohort data indicated poor performance of PFT as a stand-alone method for ILD screening. Lung HRCT appeared promising, but radiation is an issue. Promising biomarker data indicate the possibility of new ILD screening algorithms in SSc.

Lung Involvement in Rheumatic Disease: Introduction

Heterogeneity in the extent and progression of lung fibrosis probably reflects differences in the underlying pathogenic mechanisms. A growing understanding of the key pathogenic drivers of lung fibrosis and of CTDs (including the role of tobacco smoking) might lead to the development of more effective targeted therapies. The key goals of the clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated and how. Recent developments have moved the field forward. Several studies have provided significant advances, especially regarding HRCT-pathologic correlations, prediction of the outcome based on imaging and/or pathology, and the relevance of subtle clinical, serologic and morphologic imaging/pathological findings not fulfilling the criteria for CTDs (a condition now called ‘interstitial pneumonia with autoimmune features’). Demonstration of the efficacy of antifibrotic drugs to slow disease progression in idiopathic pulmonary fibrosis has raised hope that these or other drugs might be effective in fibrotic ILD associated with CTD and foster clinical research. This thematic series sets the scene of CTD principles for a series of papers that will focus on the investigation of lung involvement in rheumatologic disease, the investigation of CTD in ILD, biomarkers, treatment considerations, acute exacerbation and monitoring of lung involvement in rheumatologic disease from authorities in the field. Lung involvement in autoimmune rheumatic diseases is a common manifestation and a major cause of mortality and morbidity. Involvement may include lung parenchyma, airways, pleura, vessels and respiratory muscles. Main differential diagnoses include rheumatic disease manifestation, drug-induced manifestation or infection. Interstitial lung disease (ILD) in connective tissue disease (CTD) can be characterized using the classification of the idiopathic interstitial pneumonias. CTDs as a whole may develop all histologic patterns observed in idiopathic interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD seems to be most often usual interstitial pneumonia. Histologic patterns may be combined, as exemplified by the frequent association of ILD with lymphocytic bronchiolitis in rheumatoid arthritis. Occasionally, lung involvement can present before the diagnosis of CTD, especially in idiopathic inflammatory myopathy and rheumatoid arthritis. In order to decrease mortality, there is a need for early, exact diagnosis and effective treatment. Cooperation of experienced physicians for possible complications, including infections, drug-induced adverse effects, direct or indirect pulmonary or cardiovascular complications, is of paramount importance for the best management. Published online: June 24, 2015

Interstitial lung disease in connective tissue disease--mechanisms and management.

Pulmonary complications are an important extra-articular feature of autoimmune rheumatic diseases and a major cause of mortality. The underlying pathogenesis probably involves multiple cellular compartments, including the epithelium, lung fibroblasts, and the innate and adaptive immune system. Heterogeneity in the extent and progression of lung fibrosis probably reflects differences in underlying pathogenic mechanisms. Growing understanding of the key pathogenic drivers of lung fibrosis might lead to the development of more effective targeted therapies to replicate the treatment advances in other aspects of these diseases. Interstitial lung disease (ILD) in connective tissue disease (CTD) is characterized using the classification of the idiopathic interstitial pneumonias. Systemic sclerosis is most frequently associated with ILD and, in most of these patients, ILD manifests as a histological pattern of nonspecific interstitial pneumonia. Conversely, in rheumatoid arthritis, the pattern of ILD is most often usual interstitial pneumonia. The key goals of clinical assessment of patients with both ILD and CTD are the detection of ILD and prognostic evaluation to determine which patients should be treated. Data from treatment trials in systemic sclerosis support the use of immunosuppressive therapy, with the treatment benefit largely relating to the prevention of progression of lung disease.

Screening for interstitial lung disease in systemic sclerosis: performance of high-resolution CT with limited number of slices: a prospective study

ObjectivesEarly diagnosis of interstitial lung disease (ILD), currently the main cause of death in systemic sclerosis (SSc), is needed. The gold standard is high-resolution CT (HRCT) of the chest, but...

Standard and pocket-size lung ultrasound devices can detect interstitial lung disease in rheumatoid arthritis patients

Interstitial lung disease (ILD) is a frequent extra-articular manifestation of RA associated with increased mortality. High-resolution CT (HRCT) is used for diagnosis and follow-up, but its accuracy is counterbalanced by high costs and radiological risk. In the presence of ILD, lung US (LUS) detects vertical artefacts called B-lines. The aims of the present study were to evaluate the accuracy of LUS in the diagnosis of ILD in RA and to validate the use of a pocket-size US device (PS-USD) as a screening tool. LUS was performed with standard equipment by a trained physician through longitudinal scans following anatomical lines: 72 segments were considered (28 anteriorly and 44 posteriorly) and B-lines were counted in each segment. A B-lines score >10 identified a positive examination (presence of ILD). A second LUS session for positive/negative judgment was performed by a short-trained physician using a PS-USD. Thirty-nine patients were studied. The sensitivity and specificity of standard LUS vs HRCT were 92% and 56%, respectively. The B-line score was significantly correlated with HRCT score (r = 0.806). A total of 29 patients were studied with a PS-USD. Sensitivity and specificity for PS-USD vs HRCT were 89% and 50%. The sensitivity of LUS in the detection of ILD supports its use as a screening test for ILD in RA patients, even in the ambulatory setting with a PS-USD. The strong correlation between echographic and HRCT scores indicates LUS is a valid tool for grading and follow-up of ILD.