Low-Normal Lung Volume Correlates With Pulmonary Hypertension in Fibrotic Idiopathic Interstitial Pneumonia: Computer-Aided 3D Quantitative Analysis of Chest CT

2014 ◽  
Vol 203 (2) ◽  
pp. W166-W173 ◽  
Author(s):  
Tae Iwasawa ◽  
Shingo Kato ◽  
Takashi Ogura ◽  
Yuka Kusakawa ◽  
Shinichiro Iso ◽  
...  
2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 687.1-687
Author(s):  
K. Ninagawa ◽  
M. Kato ◽  
M. Kono ◽  
Y. Fujieda ◽  
K. Oku ◽  
...  

Background:Group 1 and 3 pulmonary hypertension (PH) develop through different pathological mechanisms but have similar hemodynamic abnormalities. Systemic sclerosis (SSc) is associated with both pulmonary vascular disease (PVD) and interstitial lung disease (ILD), making it challenging to differentiate group 1 and 3 PH in those patients. A previous study using quantitative evaluation of chest computed tomography (CT) demonstrated that normal lung volume was inversely correlated with mean pulmonary arterial pressure (mPAP) in patients with group 3 PH (1).Objectives:In this study, we aimed to assess the dominance of PVD or ILD in SSc patients by quantitative evaluation of chest CT and to evaluate each characteristic.Methods:A total of 76 SSc patients who underwent right heart catheterization (RHC) were included. Chest CT was evaluated by using a software (Synapse Vincent Ver.3.0, Fujifilm) which quantified normal and total area of the lung. Then, we calculated abnormal area by drawing normal area from total area in the lung (%). Pulmonary function test (PFT) and serum biomarkers, such as KL-6 and LDH, were also evaluated. The dominance of PVD or ILD was defined as divergent or parallel change between the first and last assessments, respectively, in mPAP and abnormal area in the lung calculated using the software. Increase or decrease by over 10% in the last assessment compared to the first assessment was considered as a significant change in mPAP or abnormal area in the lung. P values were calculated by Mann-Whitney U test, and correlation coefficients were calculated by direct regression variance.Results:The median [range] values of mPAP and abnormal area in the lung at baseline were 23 [9-65] mmHg and 30.2 [0-100] %, respectively. Of 37 SSc and PH patients, 18 were defined as having PVD dominance while 19 as ILD dominance. Abnormal area in the lung at baseline was greater in patients with ILD dominance compared to those with PVD dominance (39.1 [16.3-98.3] v.s. 14.0 [0-99] %, p=0.002), whereas mPAP was higher in patients with PVD dominance than those with ILD dominance (42.5 [23.0-65.0] v.s. 26.0 [16.0-42.0] mmHg, p=0.002). PFT parameters including forced vital capacity were not different between the two groups. The ratio of mPAP/KL-6 showed a great difference between the two groups with its significant elevation in patients with PVD dominance (p=0.007).Conclusion:Quantitative evaluation of chest CT showed great efficiency in differentiating the dominance of PVD or ILD in patients with SSc and PH. In addition, the ratio of mPAP/KL-6 may easily be used as a parameter for dominance evaluation.References:[1]Iwasawa T, Kato S, Ogura T, Kusakawa Y, Iso S, Baba T, et al. Low-normal lung volume correlates with pulmonary hypertension in fibrotic idiopathic interstitial pneumonia: computer-aided 3D quantitative analysis of chest CT. AJR Am J Roentgenol. 2014;203(2):W166-73.Disclosure of Interests:None declared


Author(s):  
Huai-Qing LOU ◽  
Chun-Xia HUANG ◽  
Guang-Yi LI ◽  
Ping LI ◽  
Shou-Ming ZHANG ◽  
...  

Background: Interstitial lung disease (ILD) is a category of chronic lung diseases with more than 200 subtypes. Idiopathic interstitial pneumonia (IIP), systemic sclerosis (SSc) ILD, and familial interstitial pneumonia (FIP) are three major groups of lung diseases with different causes or with unknown causes. Mucin5B (MUC5B) belongs to the mucin family, which contribute to the lubricating and viscoelastic properties of the whole saliva, normal lung mucus, and cervical mucus. The association between MUC5B rs35705950 and ILDs risks has been widely studied. However, the results were inconclusive and inconsistent. Methods: In the present meta-analysis, the database PubMed, Embase, Cochrane Central Register of Controlled Trials, CNKI and Chinese Biomedical Literature Database were searched till Aug 20th, 2018. Overall 16 publications with 28 studies, 76345 cases and 18402 controls were included. Results: The results indicated a significant increase of overall IIP risk for TT genotype and T allele of the rs35705950 in all genetic models (TT vs GG, OR=9.11; TT vs GT+TT, OR=5.80; GT+TT vs GG, OR=4.34; T vs G, OR=4.03. P<0.0001). Subgroup analysis by subtypes of IIP revealed higher risks of TT genotype and T allele for IPF and iNSIP (P<0.05). A significant increase of FIP risk was also found for the TT genotype and T allele of the rs35705950 (TT vs GG, OR=17.08; GT+TT vs GG, OR=6.02; T vs G, OR=1.64.P<0.05). Conclusion: No significant relations existed between the rs35705950 and SSc-ILD risks. MUC5B rs35705950 might be a predictor for the susceptibility of IIP and FIP.


2019 ◽  
Vol 7 (9) ◽  
pp. 780-790 ◽  
Author(s):  
Steven D Nathan ◽  
Jürgen Behr ◽  
Harold R Collard ◽  
Vincent Cottin ◽  
Marius M Hoeper ◽  
...  

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