Evaluation of Regional Lung Function in Pulmonary Fibrosis with Xenon-129 MRI

Idiopathic pulmonary fibrosis, a pattern of interstitial lung disease, is often clinically unpredictable in its progression. This paper presents hyperpolarized Xenon-129 chemical shift imaging as a noninvasive, nonradioactive method of probing lung physiology as well as anatomy to monitor subtle changes in subjects with IPF. Twenty subjects, nine healthy and eleven IPF, underwent HP Xe-129 ventilation MRI and 3D-SBCSI. Spirometry was performed on all subjects before imaging, and DLCO and hematocrit were measured in IPF subjects after imaging. Images were post-processed in MATLAB and segmented using ANTs. IPF subjects exhibited, on average, higher Tissue/Gas ratios and lower RBC/Gas ratios compared with healthy subjects, and quantitative maps were more heterogeneous in IPF subjects. The higher ratios are likely due to fibrosis and thickening of the pulmonary interstitium. T2* relaxation was longer in IPF subjects and corresponded with hematocrit scores, although the mechanism is not well understood. A lower chemical shift in the red blood cell spectroscopic peak correlated well with a higher Tissue/RBC ratio and may be explained by reduced blood oxygenation. Tissue/RBC also correlated well, spatially, with areas of fibrosis in HRCT images. These results may help us understand the underlying mechanism behind gas exchange impairment and disease progression.

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NOVEL X-RAY VELOCIMETRY-BASED QUANTITATIVE REGIONAL LUNG FUNCTION ANALYSIS TO ASSESS RISK OF RADIATION-INDUCED PNEUMONITIS AND PULMONARY FIBROSIS

CHEST Journal ◽

10.1016/j.chest.2019.08.315 ◽

2019 ◽

Vol 156 (4) ◽

pp. A2264-A2265

Author(s):

Andreas Fouras ◽

Stephen Shiao ◽

David Wenger ◽

Alex Winnett ◽

Chandni Doshi ◽

...

Keyword(s):

Lung Function ◽

Pulmonary Fibrosis ◽

Function Analysis ◽

X Ray ◽

Regional Lung Function ◽

Regional Lung ◽

Radiation Induced

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Volatile Organic Compounds in Exhaled Breath of Idiopathic Pulmonary Fibrosis for Discrimination from Healthy Subjects

Lung ◽

10.1007/s00408-017-9979-3 ◽

2017 ◽

Vol 195 (2) ◽

pp. 247-254 ◽

Cited By ~ 11

Author(s):

Yu-ichi Yamada ◽

Gen Yamada ◽

Mitsuo Otsuka ◽

Hirotaka Nishikiori ◽

Kimiyuki Ikeda ◽

...

Keyword(s):

Volatile Organic Compounds ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Organic Compounds ◽

Healthy Subjects ◽

Exhaled Breath ◽

Volatile Organic

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Clinicoserological features of antisynthetase syndrome (ASyS)-associated interstitial lung disease presenting to respiratory services: comparison with idiopathic pulmonary fibrosis and ASyS diagnosed in rheumatology services

BMJ Open Respiratory Research ◽

10.1136/bmjresp-2020-000829 ◽

2021 ◽

Vol 8 (1) ◽

pp. e000829

Author(s):

Shaney L Barratt ◽

Havra H Adamali ◽

Caroline Cotton ◽

Ben Mulhearn ◽

Hina Iftikhar ◽

...

Keyword(s):

Interstitial Lung Disease ◽

Idiopathic Pulmonary Fibrosis ◽

Pulmonary Fibrosis ◽

Lung Disease ◽

Trna Synthetase ◽

Musculoskeletal Symptoms ◽

Antisynthetase Syndrome ◽

Cytoplasmic Staining ◽

Phenotypic Differences ◽

Lung Physiology

IntroductionAntisynthetase syndrome (ASyS) is a rare autoimmune connective tissue disease (CTD), associated with autoantibodies targeting tRNA synthetase enzymes, that can present to respiratory (interstitial lung disease (ILD)) or rheumatology (myositis, inflammatory arthritis and systemic features) services. The therapeutic management of CTD-associated ILD and idiopathic pulmonary fibrosis (IPF) differs widely, thus accurate diagnosis is essential.MethodsWe undertook a retrospective, multicentre observational cohort study designed to (1) evaluate differences between ASyS-associated ILD with IPF, (2) phenotypic differences in patients with ASyS-ILD presenting to respiratory versus rheumatology services, (3) differences in outcomes between ASySassociated with Jo-1 versus non-Jo-1 autoantibodies and (4) compare long-term outcomes between these groups.ResultsWe identified 76 patients with ASyS-ILD and 78 with IPF. Patients with ASyS were younger at presentation (57 vs 77 years, p<0.001) with a female predominance (57% vs 33%, p=0.006) compared with IPF. Cytoplasmic staining on indirect immunofluorescence was a differentiating factor between ASyS and IPF (71% vs 0%, p<0.0001). Patients with ASyS presenting initially to respiratory services (n=52) had a higher prevalence of ASyS non-Jo-1 antibodies and significantly fewer musculoskeletal symptoms/biochemical evidence of myositis, compared with those presenting to rheumatology services (p<0.05), although lung physiology was similar in both groups. There were no differences in high-resolution CT appearances or outcomes in those with Jo-1 versus non-Jo-1 ASyS-ILD.ConclusionsExtended autoimmune serology is needed to evaluate for ASyS autoantibodies in patients presenting with ILD, particularly in younger female patients. Musculoskeletal involvement is common in ASyS (typically Jo-1 autoantibodies) presenting to rheumatology but the burden of ILD is similar to those presenting to respiratory medicine.

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