naa a22 4500 510748023 CHVBK 20180411083054.0 cr unu---uuuuu 180411e20131201xx s 000 0 eng 10.1007/s11547-013-0934-1 doi (NATIONALLICENCE)springer-10.1007/s11547-013-0934-1 Pulmonary hypertension and systemic sclerosis: the role of high-resolution computed tomography [Elektronische Daten] [Maurizio Zompatori, Maria Leone, Marica Giannotta, Nazzareno Galiè, Massimiliano Palazzini, Maria Reggiani, Lea Bono, Gian Pollini] Ipertensione polmonare e sclerodermia: il ruolo della TC ad alta risoluzione Purpose: This study sought to evaluate, by means of a retrospective analysis, the relationship between pulmonary hypertension (PH) and fibrotic interstitial lung disease (ILD) in a population of 43 patients affected by systemic sclerosis. In addition, we assessed the role of high-resolution computed tomography (HRCT) in the diagnostic and therapeutic pathway for such patients. Materials and methods: Forty-three patients affected by progressive systemic sclerosis (PSS) and PH underwent functional, haemodynamic and HRCT evaluations between November 2001 and May 2011. Functional evaluation was performed through respiratory function testing and spirometry. Haemodynamic assessment was done with transthoracic echocardiogram (ECG) and right heart catheterisation. Semiquantitative evaluation of radiological involvement was performed using different measures: visual score, Goh's score, Wells' score and Warrick's score. Results: Of 43 patients, 16 showed fibrotic ILD after a HRCT evaluation. The main pattern of ILD was identified with nonspecific interstitial pneumonia (NSIP). Our study also evaluated the relationship among radiological, functional and haemodynamic indexes; different correlations were found to be significant, in particular, Wells' and visual scores ≥40% correlated significantly with diffusion capacity for carbon monoxide (DLCO); Wells' score also correlated significantly with percentage of predicted forced vital capacity (FVC), percentage of predicted forced expiratory volume in 1 s (FEV1) and composite physiologic index (CPI). If presence of Inoesophagopathy evaluated with HRCT was included, this sign correlated significantly both with DLCO (p=0.03) and mean pulmonary arterial pressure (mPAP) (p=0.03), considering total population. Conclusions: Our findings confirm the diagnostic role of HRCT in evaluating the extent of lung damage in systemic sclerosis. Furthermore, a significant correlation between mPAP and esophagopathy suggests a possible positive predictive value of this sign in identifying a subphenotypic category of patients affected by systemic sclerosis in whom we can find a more severe visceral impairment and a more frequent vascular involvement. Springer-Verlag, 2013 Systemic sclerosis nationallicence Pulmonary hypertension nationallicence Interstitial lung disease nationallicence High-resolution computed tomography nationallicence esophagopathy nationallicence Sclerosi sistemica nationallicence Ipertensione polmonare nationallicence Interstiziopatia polmonare nationallicence HRCT nationallicence Esofagopatia nationallicence Zompatori Maurizio Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut Leone Maria Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut Giannotta Marica Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut Galiè Nazzareno Institute of Cardiology, University of Bologna, Bologna, Italy aut Palazzini Massimiliano Institute of Cardiology, University of Bologna, Bologna, Italy aut Reggiani Maria Department of Cardiovascular Diseases, University of Bologna, Bologna, Italy aut Bono Lea Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut Pollini Gian Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut La radiologia medica Springer-Verlag 118/8(2013-12-01), 1360-1372 0033-8362 118:8<1360 2013 118 11547 https://doi.org/10.1007/s11547-013-0934-1 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s11547-013-0934-1 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Zompatori Maurizio Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut NATIONALLICENCE 700 1- Leone Maria Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut NATIONALLICENCE 700 1- Giannotta Marica Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut NATIONALLICENCE 700 1- Galiè Nazzareno Institute of Cardiology, University of Bologna, Bologna, Italy aut NATIONALLICENCE 700 1- Palazzini Massimiliano Institute of Cardiology, University of Bologna, Bologna, Italy aut NATIONALLICENCE 700 1- Reggiani Maria Department of Cardiovascular Diseases, University of Bologna, Bologna, Italy aut NATIONALLICENCE 700 1- Bono Lea Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut NATIONALLICENCE 700 1- Pollini Gian Department of Radiology, Ospedale Sant'Orsola, Bologna, Italy aut NATIONALLICENCE 773 0- La radiologia medica Springer-Verlag 118/8(2013-12-01), 1360-1372 0033-8362 118:8<1360 2013 118 11547 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer