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Keywords: COLDICE; cryobiopsy; idiopathic pulmonary fibrosis; interstitial lung disease; telomeres EN COLDICE cryobiopsy idiopathic pulmonary fibrosis interstitial lung disease telomeres 756 763 8 06/22/20 20200701 NES 200701 Abbreviations CT computed tomography DL CO diffusion capacity of the lung for carbon monoxide FVC forced vital capacity ILA interstitial lung abnormality ILD interstitial lung disease IPF idiopathic pulmonary fibrosis MRCI Medication Regimen Complexity Index PrePF Preclinical pulmonary fibrosis SLB surgical lung biopsy TB tuberculosis TBLC transbronchial lung cryobiopsy Summary of key points Antifibrotic therapy has been proven to be effective in the treatment of progressive fibrosing lung diseases other than IPF. While the increased risk of sarcoidosis in patients with TB persisted throughout the 15-year follow-up period, the risk of developing TB in sarcoid patients was increased only in the first year after diagnosis. Silicosis, a severe occupational lung disease, is sometimes misdiagnosed as sarcoidosis due to imaging similarities, including centrilobular nodularity, bilateral airspace opacification (albeit unlike sarcoid often lower predominant) and calcified lymphadenopathy.[23] There remain no effective treatment options for this entirely avoidable lung disease, now a source of growing international concern.[24] IMPACT OF ILD ON QUALITY OF LIFE Key Points Patients continue to report deficiencies in the quality of their care with specific reference to provision of information, drug side effect management and access to specialist care. Polypharmacy is a common burden in patients with ILD, with the greatest increase seen in IPF patients following initiation of antifibrotic therapy. [Extracted from the article]

Interstitial lung disease (ILD) remains a major cause of morbidity and mortality in systemic sclerosis (SSc). Study aim is to characterize and quantify SSc-ILD by using Computer-Aided Lung Informatics for Pathology Evaluation and Rating (CALIPER). Secondly, our objective is to evaluate which radiological pattern is predictive of lung function decline at 12 months follow-up. In the prospective study (IRB 5435), 66 SSc patients underwent high-resolution computerized tomography (HRCT) at baseline. HRCT was performed according to standard protocol using a CT 64GE light speed VCT power scanner. CALIPER classified lung parenchyma on volume units. Every volume unit was classified into radiological parenchymal patterns (honeycombing, reticular and ground glass). Pulmonary function tests (PFTs) were performed at baseline and after 12 months of follow-up. Cigarette smoking and other lung diseases unrelated to SSc are exclusion criteria. CALIPER analysis showed normal lung parenchyma 87.4 ± 9.8%, ground glass 2.8 ± 5.3%, reticular 4 ± 5.7%, and honeycombing 1 ± 1%. In multiple regression analysis, FEV1 (p < 0.0001), FVC (p = 0.001), and DLCO (p < 0.0001) measurements at baseline showed a negative correlation with the reticular pattern percentage. At follow-up, DLCO reduction showed a positive correlation (p < 0.001) with the percentage of ground glass pattern (r = 0.33, beta coefficient = 0.51). In the ROC curve analysis, ground glass score is a good predictor (0.75, p = 0.009; 95% CI 0.59–0.91) of DLCO worsening, defined as a decrease of more than 10% of DLCO. Using a cutoff ≥ 4.5 for ground glass score, the RR for DLCO worsening is 6.8 (p < 0.01; 95% CI 1.6–29.2). The results of this study show that CALIPER is useful not only for quantifying lung damage but also for assessing worsening PFTs, but larger studies are needed to confirm these preliminary data. Key Points • At baseline reticular pattern showed negative correlation with PFTs • At follow-up ground glass pattern predicts worsening of DLCO • CALIPER is a useful to quantify lung damage [ABSTRACT FROM AUTHOR]

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Background and objective: This study aimed to investigate whether quantitative lung vessel morphology determined by a new fully automated algorithm is associated with functional indices in idiopathic pulmonary fibrosis (IPF). Methods: A total of 152 IPF patients had vessel volume, density, tortuosity and heterogeneity quantified from computed tomography (CT) images by a fully automated algorithm. Separate quantitation of vessel metrics in pulmonary arteries and veins was performed in 106 patients. Results were evaluated against readouts from lung function tests. Results: Normalized vessel volume expressed as a percentage of total lung volume was moderately correlated with functional indices on univariable linear regression analysis: forced vital capacity (R2 = 0.27, P < 1 × 10−6), diffusion capacity for carbon monoxide (DLCO; R2 = 0.12, P = 3 × 10−5), total lung capacity (TLC; R2 = 0.45, P < 1 × 10−6) and composite physiologic index (CPI; R2 = 0.28, P < 1 × 10−6). Normalized vessel volume was correlated with vessel density but not with vessel heterogeneity. Quantitatively derived vessel metrics (and artery and vein subdivision scores) were not significantly linked with the transfer factor for carbon monoxide (KCO), and only weakly with DLCO. On multivariable linear regression analysis, normalized vessel volume and vessel heterogeneity were independently linked with DLCO, TLC and CPI indicating that they capture different aspects of lung damage. Artery–vein separation provided no additional information beyond that captured in the whole vasculature. Conclusion: Our study confirms previous observations of links between vessel volume and functional measures of disease severity in IPF using a new vessel quantitation tool. Additionally, the new tool shows independent linkages of normalized vessel volume and vessel heterogeneity with functional indices. Quantitative vessel metrics do not appear to reflect vasculopathic damage in IPF. [ABSTRACT FROM AUTHOR]

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