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
FRI0453 Hospitalisation and survival analysis in systemic sclerosis patients with concomitant or isolated pulmonary hypertension and interstitial lung disease in the multiethnic scleroderma cohort singapore
