Sa141 REDUCED PROXIMAL ESOPHAGEAL CONTRACTILITY AND MUCOSAL INTEGRITY ARE INDEPENDENT PREDICTORS OF POOR PULMONARY FUNCTION IN INTERSTITIAL LUNG DISEASE

Background. Although digital clubbing is a common presentation in patients with interstitial lung disease (ILD), little has been reported regarding its role in assessing patients with ILD. This study evaluated patients with ILD for the presence of clubbing and investigated its association with clinical data. Methods. We evaluated patients with ILD who visited the teaching hospital at which the study was conducted, between October 2014 and January 2015. Clubbing, evaluated using a Vernier caliper for individual patients, was defined as a phalangeal depth ratio > 1. We examined the association of clubbing with clinical data. Results. Of 102 patients with ILD, we identified 17 (16.7%) with clubbing. The partial pressure of oxygen in arterial blood was lower (65.2 ± 5.9 mmHg versus 80.2 ± 3.1 mmHg; p=0.03), serum Krebs von den Lugen-6 (KL-6) levels were higher (1495.0 ± 277.4 U/mL versus 839.1 ± 70.2 U/mL; p=0.001), and the percent predicted diffusing capacity of carbon monoxide was lower (50.0 ± 6.0 versus 73.5 ± 3.1; p=0.002) in these patients with clubbing. Conclusions. Patients with clubbing had lower oxygen levels, higher serum KL-6 levels, and lower pulmonary function than those without clubbing.

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99mTc-MIBI Lung Scintigraphy in the Assessment of Pulmonary Involvement in Interstitial Lung Disease and Its Comparison With Pulmonary Function Tests and High-Resolution Computed Tomography

Medicine ◽

10.1097/md.0000000000002082 ◽

2015 ◽

Vol 94 (47) ◽

pp. e2082 ◽

Cited By ~ 1

Author(s):

Mehrzad Bahtouee ◽

Jamshid Saberifard ◽

Hamid Javadi ◽

Iraj Nabipour ◽

Alireza Raeisi ◽

...

Keyword(s):

Computed Tomography ◽

High Resolution ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Pulmonary Involvement ◽

Lung Scintigraphy ◽

High Resolution Computed Tomography ◽

99Mtc Mibi

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Predictive Factors for the Long-Term Deterioration of Pulmonary Function in Interstitial Lung Disease Associated with Anti-Aminoacyl-tRNA Synthetase Antibodies

Respiration ◽

10.1159/000488358 ◽

2018 ◽

Vol 96 (3) ◽

pp. 210-221 ◽

Cited By ~ 8

Author(s):

Hideaki Yamakawa ◽

Eri Hagiwara ◽

Hideya Kitamura ◽

Tae Iwasawa ◽

Ryota Otoshi ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Predictive Factors ◽

Trna Synthetase ◽

Aminoacyl Trna Synthetase

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Lung ultrasound B-lines and serum KL-6 correlate with the severity of idiopathic inflammatory myositis-associated interstitial lung disease

Rheumatology ◽

10.1093/rheumatology/kez571 ◽

2019 ◽

Vol 59 (8) ◽

pp. 2024-2029 ◽

Cited By ~ 1

Author(s):

Yukai Wang ◽

Shaoqi Chen ◽

Jianqun Lin ◽

Xuezhen Xie ◽

Shijian Hu ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Lung Ultrasound ◽

Pulmonary Function Tests ◽

Forced Expiratory Volume ◽

Inflammatory Myositis ◽

Idiopathic Inflammatory Myositis ◽

Function Tests ◽

B Lines

Abstract Objective Idiopathic inflammatory myositis-associated interstitial lung disease (IIM-ILD) significantly increases morbidity and mortality. Lung ultrasound B-lines and Krebs von den Lungen-6 (KL-6) are identified as new sonographic and serum markers of ILD, respectively. The aim of our work was to assess the role of B-lines and KL-6 as markers of the severity of IIM-ILD. For this purpose, the correlation among B-lines score, serum KL-6 levels, high-resolution CT (HRCT) score, and pulmonary function tests were investigated in IIM-ILD patients. Methods Thirty-eight patients with IIM-ILD underwent chest HRCT scans, lung ultrasound and pulmonary function tests (independently performed within 1 week) examination. To assess severity and extent of ILD at HRCT, the Warrick score was used. The B-lines score denoting the extension of ILD was calculated by summing the number of B-lines on a total of 50 scanning sites. Serum KL-6 levels (U/ml) was measured by chemiluminescent enzyme immunoassay. Results A significant correlation was found between the B-lines score and serum KL-6 levels (r = 0.43, P < 0.01), and between the Warrick score and serum KL-6 levels (r = 0.45, P < 0.01). A positive correlation between B-lines score and the Warrick score (r = 0.87, P < 0.0001) was also confirmed. Both B-lines score and KL-6 levels inversely correlated to diffusion capacity for carbon monoxide (r = −0.77, P < 0.0001 and r = −0.42, P < 0.05, respectively) and total lung capacity (r = −0.73, P < 0.0001 and r = −0.36, P < 0.05, respectively). Moreover, B-lines correlated inversely with forced vital capacity (r = −0.73, P < 0.0001), forced expiratory volume in 1 s (r = −0.69, P < 0.0001). Conclusion B-lines score and serum KL-6 levels correlate with HRCT findings and pulmonary function tests, supporting their use as measures of IIM-ILD severity.

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Applicability of radiomics in interstitial lung disease associated with systemic sclerosis: proof of concept

European Radiology ◽

10.1007/s00330-020-07293-8 ◽

2020 ◽

Cited By ~ 1

Author(s):

K. Martini ◽

B. Baessler ◽

M. Bogowicz ◽

C. Blüthgen ◽

M. Mannil ◽

...

Keyword(s):

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Data Augmentation ◽

Visual Analysis ◽

Area Under The Curve ◽

Information Criterion ◽

Visual Assessment ◽

High Resolution Computed Tomography

Abstract Objective To retrospectively evaluate if texture-based radiomics features are able to detect interstitial lung disease (ILD) and to distinguish between the different disease stages in patients with systemic sclerosis (SSc) in comparison with mere visual analysis of high-resolution computed tomography (HRCT). Methods Sixty patients (46 females, median age 56 years) with SSc who underwent HRCT of the thorax were retrospectively analyzed. Visual analysis was performed by two radiologists for the presence of ILD features. Gender, age, and pulmonary function (GAP) stage was calculated from clinical data (gender, age, pulmonary function test). Data augmentation was performed and the balanced dataset was split into a training (70%) and a testing dataset (30%). For selecting variables that allow classification of the GAP stage, single and multiple logistic regression models were fitted and compared by using the Akaike information criterion (AIC). Diagnostic accuracy was evaluated from the area under the curve (AUC) from receiver operating characteristic (ROC) analyses, and diagnostic sensitivity and specificity were calculated. Results Values for some radiomics features were significantly lower (p < 0.05) and those of other radiomics features were significantly higher (p = 0.001) in patients with GAP2 compared with those in patients with GAP1. The combination of two specific radiomics features in a multivariable model resulted in the lowest AIC of 10.73 with an AUC of 0.96, 84% sensitivity, and 99% specificity. Visual assessment of fibrosis was inferior in predicting individual GAP stages (AUC 0.86; 83% sensitivity; 74% specificity). Conclusion The correlation of radiomics with GAP stage, but not with the visually defined features of ILD-HRCT, implies that radiomics might capture features indicating severity of SSc-ILD on HRCT, which are not recognized by visual analysis. Key Points • Radiomics features can predict GAP stage with a sensitivity of 84% and a specificity of almost 100%. • Extent of fibrosis on HRCT and a combined model of different visual HRCT-ILD features perform worse in predicting GAP stage. • The correlation of radiomics with GAP stage, but not with the visually defined features of ILD-HRCT, implies that radiomics might capture features on HRCT, which are not recognized by visual analysis.

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Frailty Is Associated with Decreased Pulmonary Function in Interstitial Lung Disease

10.1164/ajrccm-conference.2019.199.1_meetingabstracts.a7143 ◽

2019 ◽

Author(s):

S. Bhorade ◽

R. Jablonski ◽

J. Lee ◽

M. Masihi ◽

A. Ansari ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease

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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

Rheumatology ◽

10.1093/rheumatology/keaa109.028 ◽

2020 ◽

Vol 59 (Supplement_2) ◽

Author(s):

Ashraful Haque ◽

Rachael Kilding ◽

Ruth Smith ◽

Sameena Khalid ◽

Robert Sandler ◽

...

Keyword(s):

Rheumatoid Arthritis ◽

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Newly Diagnosed ◽

Tertiary Centre ◽

Function Tests ◽

Low Sensitivity ◽

New Diagnosis

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.

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Pulmonary function testing in children's interstitial lung disease

European Respiratory Review ◽

10.1183/16000617.0019-2020 ◽

2020 ◽

Vol 29 (157) ◽

pp. 200019

Author(s):

Astrid Madsen Ring ◽

Julia Carlens ◽

Andy Bush ◽

Silvia Castillo-Corullón ◽

Salvatore Fasola ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Pulmonary Function ◽

Lung Disease ◽

Pulmonary Function Tests ◽

Clinical Care ◽

Whole Body ◽

Future Research ◽

Exercise Tests ◽

Multiple Breath Washout ◽

Randomised Controlled

The use of pulmonary function tests (PFTs) has been widely described in airway diseases like asthma and cystic fibrosis, but for children's interstitial lung disease (chILD), which encompasses a broad spectrum of pathologies, the usefulness of PFTs is still undetermined, despite widespread use in adult interstitial lung disease. A literature review was initiated by the COST/Enter chILD working group aiming to describe published studies, to identify gaps in knowledge and to propose future research goals in regard to spirometry, whole-body plethysmography, infant and pre-school PFTs, measurement of diffusing capacity, multiple breath washout and cardiopulmonary exercise tests in chILD. The search revealed a limited number of papers published in the past three decades, of which the majority were descriptive and did not report pulmonary function as the main outcome.PFTs may be useful in different stages of management of children with suspected or confirmed chILD, but the chILD spectrum is diverse and includes a heterogeneous patient group in all ages. Research studies in well-defined patient cohorts are needed to establish which PFT and outcomes are most relevant for diagnosis, evaluation of disease severity and course, and monitoring individual conditions both for improvement in clinical care and as end-points in future randomised controlled trials.

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