scholarly journals SAT0553 QUANTITATIVE ANALYSIS OF IMAGING FEATURES AT CHEST CT OF PULMONARY ARTERIAL AND VENOUS COMPONENTS IN SYSTEMIC SCLEROSIS-INTERSTITIAL LUNG DISEASE (SSc-ILD).

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1234.1-1234
Author(s):  
C. Bruni ◽  
M. Occhipinti ◽  
G. Camiciottoli ◽  
M. Bartolucci ◽  
M. Pienn ◽  
...  
Keyword(s):  
Quantitative Analysis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Physiological Mechanism ◽  
Chest Ct ◽  
Ct Scans ◽  
Vascular Involvement ◽  
Imaging Features ◽  
Positive Correlation ◽  
Pulmonary Arterial

Background:Interstitial lung disease (ILD) and pulmonary arterial hypertension carry a negative impact on SSc prognosis. Chest CT is the gold standard in assessing ILD and helps in evaluating associated vascular involvement.Objectives:As qualitative analysis of CT scans is limited by low reproducibility and time constraints, we aimed at evaluating parenchymal and vascular features in SSc-ILD by quantitative analysis (QA) of CT scans and testing the relationship with clinical-functional data.Methods:We prospectively enrolled 80 patients who underwent PFTs and chest CT scan spirometry gated at TLC on the same day. Clinical, lung functional and diffusion data, as well as disability indexes were collected. CT images were analyzed by a computational platform for texture analysis of ILD patterns (CALIPER), through Imbio LTA. It quantified the extent of normal pattern (NP %), ground glass opacities (GG %), reticulation (RET %), honeycombing (HC %), total ILD extent (ILD EXT %) and hyperlucent (HL %). Low density areas, representing emphysematous area, were also quantified (LDA %). For lung vessel analysis, a software program developed by the Ludwig Boltzmann Institute for Lung Vascular Research was used. This software determined total, arterial, and venous vascular volumes (TV, AV, VV), and relative volumes (TV%, AV%, VV%), as well as density and number for total, arterial and venous vessels.Results:43/80 patients/CT scans were eligible for both software analyses, while 36/43 for arterial and venous separation. TV% and total vessel density were correlated positively with mRSS and negatively with %FVC (r=-0.537 and r=-0.382) and %TLC (r=-0.511 and r=-0.648), while vessel tortuosity correlated positively with %DLco. This was confirmed when separately analyzing arterial vessels, while VV% negatively correlated with %FVC, %TLC and %DLco. There was a positive correlation between %ILD patterns and %vascular volumes, being significant for TV%-AV%, total vessels and arterial density. Conversely, %ILD patterns were negatively correlated with VV and number of veins detected, despite positive correlation between VV% and ILD_EXT%. When clustering patients according to %FVC and %DLco with 80% normal cutoff, %FVC allowed clustering according to significantly different ILD patterns extents and vascular features, while %DLCO for vascular features only. Moreover, the consecutive addition of functional impairment and worsening of ILD (from both normal %FVC and %Dlco, to %DLco impairment only to both %FVC and %Dlco impairment), there was a significant increase in %TV, % AV and %VV, with the exception of decrease in %VV and venous density in patients with double impairment versus DLco single impairment.Conclusion:This is the first study showing in SSc a direct correlation between ILD and the increase in lung vascular volume, which is characterized by increase in arterial volume and density and reduction in venous volume and number. These results might be explained by the reduction of pulmonary volume due to fibrosis. However, also a para-physiological mechanism of redistribution of blood flow in lung areas, less involved by ILD, might be considered. Further studies on lung vessel quantification and distribution are ongoing.Disclosure of Interests:Cosimo Bruni Speakers bureau: Actelion, Eli Lilly, Mariaelena Occhipinti Consultant of: Imbio, Gianna Camiciottoli: None declared, Maurizio Bartolucci: None declared, Michael Pienn: None declared, Gemma Lepri: None declared, Alessio Fabbrizzi: None declared, Alessandra Tottoli: None declared, Giuglia Ciardi: None declared, Dilia Giuggioli: None declared, Giovanna CUOMO: None declared, Francesco Masini: None declared, Horst Olschewski: None declared, Federico Lavorini: None declared, Linda Calistri: None declared, Marco Matucci-Cerinic Grant/research support from: Actelion, MSD, Bristol-Myers Squibb, Speakers bureau: Acetelion, Lilly, Boehringer Ingelheim

scholarly journals Prognostic effects of clinical and CT imaging features on critically ill patients with interstitial lung disease hospitalized in respiratory intensive care unit

2019 ◽  
Vol 9 (1) ◽  
Author(s):  
Shenyun Shi ◽  
Yonglong Xiao ◽  
Xiaohua Qiu ◽  
Yan Li ◽  
Yuying Qiu ◽  
...  
Keyword(s):  
Intensive Care Unit ◽  
Intensive Care ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Critically Ill ◽  
Critically Ill Patients ◽  
Chest Ct ◽  
Imaging Features ◽  
Significant Difference ◽  
Respiratory Intensive Care Unit

AbstractThe study aimed to evaluate the clinical and imaging features of critically ill patients with interstitial lung disease (ILD) treated in respiratory intensive care unit (RICU) and assess the prognostic effects of these factors. A total of 160 severe ILD patients admitted to the RICU were finally enrolled in this study. The clinical, imaging and follow-up data of them were studied retrospectively. The in-hospital mortality and total mortality were 43.1% and 63.8% respectively. By multivariate cox regression analysis, shock (OR = 2.39, P = 0.004), pulmonary fibrosis on CT (OR = 2.85, P = 0.002) and non-invasive ventilation (OR = 1.86, P = 0.037) were harmful factors to survivals of critically ill patients with ILD. In contrast, oxygenation index (OR = 0.99, P = 0.028), conventional oxygen therapy (OR = 0.59, P = 0.048) and β-lactam antibiotics use (OR = 0.51, P = 0.004) were protective factors. There is significant difference of survivals between patients with and without fibrosing ILD on CT (Log-rank, p = 0.001). The prognosis of critically ill patients with ILD was poor. Shock, respiratory failure and fibrosing signs on chest CT affected the prognosis. Chest CT was considered as a valuable tool to indicate the prognosis.

scholarly journals COVID-19 or clinical amyopathic dermatomyositis associated rapidly progressive interstitial lung disease? A case report

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Mengke Cao ◽  
Shuangshuang Zhang ◽  
Dejie Chu ◽  
Ming Xiao ◽  
Xiaohong Liu ◽  
...  
Keyword(s):  
Case Report ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Poor Prognosis ◽  
Chest Ct ◽  
Lung Damage ◽  
Ct Scans ◽  
Amyopathic Dermatomyositis ◽  
Case Presentation ◽  
Case Diagnosis

Abstract Background Coronavirus disease 2019 (COVID-19) has reach pandemic proportions globally. For patients with symptoms of fever and cough accompanied by rapid lung damage progression, COVID-19 needs to be distinguished from interstitial lung disease (ILD) attributed to connective tissue disease (CTD), especially dermatomyositis (DM)/clinical amyopathic dermatomyositis (CADM) associated rapidly progressive interstitial lung disease (RP-ILD). Case presentation We report a case of a woman observed with fever, cough, and rapid lung damage during the epidemic. The patient had a suspicious epidemiological history, and her chest CT scans showed lung damage similar to that caused by COVID-19, but anti-Ro52 antibody was strongly positive. She was diagnosed with CADM associated RP-ILD and died 1 month later. Conclusions During the COVID-19 epidemic, it is critical to carefully assess patients with CTD related ILD, especially RP-ILD associated with CADM. Repeated nucleic acid tests for COVID-19 are necessary to achieve accurate case diagnosis. High-resolution CT (HRCT) of the chest is presently deemed an inefficient technique to distinguishing between COVID-19 and CADM associated RP-ILD. The characteristic rashes of dermatomyositis require careful observation and can often provide diagnostic clues. For patients with CADM, a high titers of anti-Ro52 antibody may be related to the pathogenesis of RP-ILD, suggesting a poor prognosis.

scholarly journals SAT0306 SEMIQUANTITATIVE AND QUANTITATIVE ANALYSIS OF LUNG CT IN THE ASSESSMENT OF INTERSTITIAL LUNG DISEASE IN IDIOPATHIC INFLAMMATORY MYOPATHIES WITH A FOCUS ON ANTISYNTHETASE.

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1098.2-1098
Author(s):  
S. Barsotti ◽  
C. Roncella ◽  
A. Valentini ◽  
L. Cavagna ◽  
R. Castellana ◽  
...  
Keyword(s):  
Quantitative Analysis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Special Focus ◽  
Idiopathic Inflammatory Myopathies ◽  
Inflammatory Myopathies ◽  
Increased Risk ◽  
Group 2 ◽  
Lung Ct ◽  
Group 1

Background:Interstitial lung disease (ILD), is common in patients with idiopathic inflammatory myopathies (IIM) and strongly impact on patients’ morbidity and mortality. Patients with anti-aminoacyl-transfer RNA-synthetases (anti-ARS) antibodies are associated with an increased risk of ILD.Objectives:Defining the radiological characteristics of IIM patients, with special focus on serological groups, through qualitative, semiquantitative and quantitative analysis of lung CT.Methods:This was a prospective study conducted from 2016 to 2019. Ninety-eight IIM patients (35 men, 63 women) were included. Myositis specific autoantibodies (MSA) were assessed with Myositis Prophyle III (Euroimmune, Lubeck).Each patient had a baseline CT; the total score of Warrick (WS) was obtained at semiquantitative analysis. The radiological scores ILD% (interstitial lung disease %) and PVRS% (pulmonary vascular related structure) were the result of quantitative analysis in 61 patients (CALIPER). Pulmonary function tests (PFTs) included TLC%, FVC% and DLCO% (65 patients). The analysis was conducted in the whole group and divided in subgroups based on their MSA pattern: in particular anti-ARS (Group 1) and patients negative to MSA (Group 2) were analysed.Results:Positive correlations between ILD% and PVRS% (Rho=0.916; ρ=0.000), WS and ILD% (Rho=0.663; ρ=0.000) and WS and PVRS% (Rho=0.637; ρ<0.001) were found.The most relevant inverse correlations were found between ILD% and DLCO% (Rho=-0.590; ρ=0.001), PVRS% and DLCO% (Rho=-0.549; ρ<0.001) and WS and DLCO% (Rho=-0.471; ρ<0.001).Statistically significant higher values of WS, ILD% and PVRS% were found in Group 1 (WS=15, ILD%=11 and PVRS%=3.5), compared to Group 2 (WS=2.5, ILD%=0.84 and PVRS%=2.2). NSIP pattern resulted dominant represented in the two groups (80% Group 1, 75% Group 2). No statistically significant differences of DLCO%, FVC% and TLCO% were found.Conclusion:The inverse correlations between the radiological scores and the functional data TLC% and DLCO% (ρ<0.001) confirm the role of lung CT in the clinical management of ILD in IIM patients, and may represent a promising tool for clinical trials. For the first time anti-ARS and serological negative patients were defined through qualitative, semiquantitative and quantitative analysis of lung CT. Further study should be conducted in order to define the prognostic value of the quantitative analysis of lung CT in the follow up of IIM patients.Disclosure of Interests:None declared

scholarly journals Patients with systemic sclerosis-associated pulmonary arterial hypertension express a genomic signature distinct from patients with interstitial lung disease

2018 ◽  
Vol 3 (3) ◽  
pp. 242-248 ◽  
Author(s):  
Matthew Moll ◽  
Romy B Christmann ◽  
Yuqing Zhang ◽  
Michael L Whitfield ◽  
Yu Mei Wang ◽  
...  
Keyword(s):  
Gene Expression ◽  
Systemic Sclerosis ◽  
Pulmonary Arterial Hypertension ◽  
Interstitial Lung Disease ◽  
Arterial Hypertension ◽  
Lung Disease ◽  
Expression Profiles ◽  
Area Under The Curve ◽  
Gene Expression Profiles ◽  
Pulmonary Arterial

Objective: Pulmonary arterial hypertension and interstitial lung disease are major causes of mortality in systemic sclerosis. We used a previously identified microarray biomarker to determine whether systemic sclerosis-pulmonary arterial hypertension and systemic sclerosis-interstitial lung disease patients demonstrate distinct gene expression profiles. Methods: Peripheral blood mononuclear cells were collected from healthy controls ( n = 10), systemic sclerosis patients without pulmonary hypertension (systemic sclerosis-no pulmonary arterial hypertension, n = 39), and systemic sclerosis-pulmonary arterial hypertension patients ( n = 21; mean pulmonary arterial pressure ≥25, pulmonary capillary wedge pressure ≤15, and pulmonary vascular resistance ≥3 Wood units) diagnosed by right heart catheterization. Systemic sclerosis-interstitial lung disease patients were defined as those with evidence of fibrosis on chest computed tomography and significant restriction (forced vital capacity <70% predicted, n = 11). Systemic sclerosis-pulmonary arterial hypertension biomarker included 69 genes selected by unbiased statistical screening of three publicly available microarray studies. RNA levels were measured by NanoString Technologies. Gene expression levels that were significantly correlated with pulmonary arterial hypertension (multiple statistical measures) were chosen as inputs into a forward selection logistic regression model. Results: When interstitial lung disease patients were included ( n = 64), four genes (S100P, CD8B1, CCL2, and TIMP1) and male sex predicted pulmonary arterial hypertension with a high level of accuracy (area under the curve = 0.83). Without interstitial lung disease patients ( n = 53), two genes (THBS1 and CD8B1) and male sex predicted pulmonary arterial hypertension with a high level of accuracy (area under the curve = 0.80). When examining systemic sclerosis patients with borderline elevated pulmonary pressures (mean pulmonary arterial pressure = 21–24 mmHg), gene expression changes closely resembled the systemic sclerosis-pulmonary arterial hypertension group, except for THBS1. Conclusion: Systemic sclerosis-pulmonary arterial hypertension and systemic sclerosis-interstitial lung disease have similar but distinct gene expression profiles. Many gene expression changes occur early in the disease course, potentially allowing early detection. THBS1 appears to be an important mediator in the development of pulmonary arterial hypertension-predominant phenotype. Further prospective investigation is warranted.

scholarly journals ‘When you hear hooves, think zebras, not horses’; two challenging cases of interstitial lung disease (ILD)

2019 ◽  
Vol 12 (2) ◽  
pp. bcr-2018-224507
Author(s):  
Marissa O’Callaghan ◽  
Aurelie Fabre ◽  
Michael Keane ◽  
Timothy J McDonnell
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Lung Fibrosis ◽  
Lung Diseases ◽  
Hypersensitivity Pneumonitis ◽  
Usual Interstitial Pneumonia ◽  
Case Series ◽  
Imaging Features ◽  
Exertional Dyspnoea ◽  
Multidisciplinary Team Meeting

Our case series describes two siblings with complex fibrosing lung diseases. The first patient was initially given a diagnosis of sarcoidosis based on imaging and exclusion of alternative diagnoses. A number of years after diagnosis, he had rapid deterioration of his disease and following surgical lung biopsy, his lung fibrosis was re-classified as chronic hypersensitivity pneumonitis (cHP) with a usual interstitial pneumonia pattern. He subsequently underwent successful lung transplantation. The second patient presented with rapidly progressing exertional dyspnoea. His bloods, imaging, bronchoalveolar lavage and histology were discussed at our multidisciplinary team meeting. His histology was most in keeping with subacute on cHP with overlapping imaging features between the two siblings. He was treated accordingly but unfortunately succumbed to his illness shortly after diagnosis. These cases highlight the difficulties differentiating between the various interstitial lung disease (ILD) subtypes and the challenges in management while also increasing awareness of familial ILD.

scholarly journals The quantitative assessment of interstitial lung disease with positron emission tomography scanning in systemic sclerosis patients

Rheumatology ◽  
2019 ◽  
Vol 59 (6) ◽  
pp. 1407-1415 ◽  
Author(s):  
Daphne M Peelen ◽  
Ben G J C Zwezerijnen ◽  
Esther J Nossent ◽  
Lilian J Meijboom ◽  
Otto S Hoekstra ◽  
...  
Keyword(s):  
High Resolution ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Fdg Pet ◽  
Quantitative Assessment ◽  
Ct Scanning ◽  
Ct Scans ◽  
Pet Ct ◽  
18F Fdg ◽  
Fdg Pet Ct

Abstract Objectives The reversibility of interstitial lung disease (ILD) in SSc is difficult to assess by current diagnostic modalities and there is clinical need for imaging techniques that allow for treatment stratification and monitoring. 18F-Fluorodeoxyglucose (FDG) PET/CT scanning may be of interest for this purpose by detection of metabolic activity in lung tissue. This study aimed to investigate the potential role of 18F-FDG PET/CT scanning for the quantitative assessment of SSc-related active ILD. Methods 18F-FDG PET/CT scans and high resolution CT scans of eight SSc patients, including five with ILD, were analysed. For comparison, reference groups were included: eight SLE patients and four primary Sjögren’s syndrome (pSS) patients, all without ILD. A total of 22 regions of interest were drawn in each patient at apical, medial and dorsobasal lung levels. 18F-FDG uptake was measured as mean standardized uptake value (SUVmean) in each region of interest. Subsequently, basal/apical (B/A) and medial/apical (M/A) ratios were calculated at patient level (B/A-p and M/A-p) and at tissue level (B/A-t and M/A-t). Results SUVmean values in dorsobasal ROIs and B/A-p ratios were increased in SSc with ILD compared with SSc without ILD (P = 0.04 and P = 0.07, respectively), SLE (P = 0.003 and P = 0.002, respectively) and pSS (P = 0.03 and P = 0.02, respectively). Increased uptake in the dorsobasal lungs and increased B/A-t ratios corresponded to both ground glass and reticulation on high resolution CT. Conclusion Semi-quantitative assessment of 18F-FDG PET/CT is able to distinguish ILD from non-affected lung tissue in SSc, suggesting that it may be used as a new biomarker for SSc-ILD disease activity.

Interstitial Lung Abnormalities in People With HIV Infection and Uninfected Controls

2020 ◽  
Vol 221 (12) ◽  
pp. 1973-1977 ◽  
Author(s):  
Andreas Ronit ◽  
Thomas Benfield ◽  
Jens Lundgren ◽  
Jørgen Vestbo ◽  
Shoaib Afzal ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Hiv Infection ◽  
Lung Disease ◽  
Chest Ct ◽  
Cross Sectional ◽  
Disease States ◽  
Ct Findings ◽  
Lung Abnormalities ◽  
Sectional Analysis ◽  
Over Time

Abstract Background Chest computed tomography (CT) findings in well-treated people with HIV infection (PWH) remain poorly characterized. Methods Cross-sectional analysis examining interstitial chest CT findings in PWH (n = 754) and uninfected controls (n = 470). Results HIV infection was independently associated with 1.82 (95% CI, 1.18–2.88) and 5.15 (95% CI, 1.72–22.2) higher adjusted odds of any interstitial lung abnormality and findings suspicious for interstitial lung disease, respectively. Conclusions HIV infection was independently associated with interstitial lung abnormalities and findings suspicious for interstitial lung disease. Whether these abnormalities develop into more recognizable disease states over time is unknown but warrants further investigation.

scholarly journals Antifibroblast antibodies from systemic sclerosis patients bind to α-enolase and are associated with interstitial lung disease

2009 ◽  
Vol 69 (2) ◽  
pp. 428-433 ◽  
Author(s):  
B Terrier ◽  
M C Tamby ◽  
L Camoin ◽  
P Guilpain ◽  
A Bérezné ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Human Fibroblasts ◽  
Target Antigen ◽  
Diffusion Capacity ◽  
Serum Igg ◽  
Normal Human ◽  
Anticentromere Antibodies ◽  
Pulmonary Arterial

Objective:To identify target antigens of antifibroblast antibodies (AFA) in systemic sclerosis (SSc) patients.Patients and Methods:In the first part, sera from 24 SSc patients (12 with pulmonary arterial hypertension (PAH) and 12 without) and 36 idiopathic PAH patients, tested in pooled sera for groups of three, were compared with a sera pool from 14 healthy controls (HC). Serum IgG reactivity was analysed by the use of a two-dimensional electrophoresis and immunoblotting technique with normal human fibroblasts antigens. In the second part, serum IgG reactivity for two groups: 158 SSc, 67 idiopathic PAH and 100 HC; and 35 SSc and 50 HC was tested against α-enolase from Saccharomyces cerevisiae and recombinant human (rHu) α-enolase, respectively, on ELISA.Results:In the first part, α-enolase was identified as a main target antigen of AFA from SSc patients. In the second part, 37/158 (23%) SSc patients, 6/67 (9%) idiopathic PAH patients and 4/100 (4%) HC (p<0.001) had anti-S cerevisiae α-enolase antibodies; 12/35 (34%) SSc patients and 3/50 (6%) HC had anti-rHu α-enolase antibodies (p = 0.001). In SSc, the presence of anti-S cerevisiae α-enolase antibodies was associated with interstitial lung disease (ILD), decreased total lung capacity (73.2% vs 89.7%; p<0.001) and diffusion capacity for carbon monoxide (47.4% vs 62.3%; p<0.001), and antitopoisomerase 1 antibodies (46% vs 21%; p = 0.005) but not anticentromere antibodies (11% vs 34%; p = 0.006). Results were similar with rHu α-enolase testing.Conclusion:In SSc, AFA recognise α-enolase and are associated with ILD and antitopoisomerase antibodies.

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