PMID- 24613044 OWN - NLM STAT- MEDLINE DCOM- 20150212 LR - 20220317 IS - 1444-2892 (Electronic) IS - 1443-9506 (Linking) VI - 23 IP - 7 DP - 2014 Jul TI - Pulmonary arterial hypertension and systemic sclerosis relation: a single centre experience. PG - 667-73 LID - S1443-9506(14)00070-5 [pii] LID - 10.1016/j.hlc.2014.02.002 [doi] AB - AIM: In systemic sclerosis (SSc), this single-centre study aimed to define the frequency and association of pulmonary arterial hypertension (PAH), occurring either alone in SSc-PAH or together with interstitial lung disease (ILD-PH). MATERIAL-METHODS: SSc cases between the years 1990-2011 were reviewed, retrospectively. Patients' clinical, laboratory findings, Modified Rodnan Skin Score and Medsger score, 6-minute walk distance (6MWD), carbon monoxide diffusion test (DLCO), echocardiography, thorax HRCT, and right heart catheterisation findings were recorded. RESULTS: One hundred and forty-one cases (F/M:124/17, diffuse cutaneous SSc (DcSSc)/limited cutaneous SSc (LcSSc): 84/57) were included in the study with the mean age of 52.70+/-15.17 years and disease duration of 107.07+/-99.44 months. PaO(2), FEV1 and FVC were lower in DcSSc (p<0.05) as compared to LcSSc, but DLCO and 6MWD did not differ significantly, between the two forms. Ground glass opacity (64.7%) and interlobular septal thickening (58.8%) were the most frequent findings on HRCT of such subjects. PAH was detected in 34 subjects (24.1%). Seven of them had SSc associated PAH (SSc-PAH) and 27 ILD-PH. Both frequencies were similar between DcSSc and LcSSc. Mean sPAP was higher in SSc-PAH. CONCLUSION: PAH was observed in approximately one fourth of patients; therefore advanced cardio-pulmonary investigation should be routinely performed in the SSc patients' management. CI - Copyright (c) 2014 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved. FAU - Demir, Nalan AU - Demir N AD - Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey. FAU - Sahin, Ali AU - Sahin A AD - Department of Rheumatology, Ankara/Turkey. FAU - Kucuksahin, Orhan AU - Kucuksahin O AD - Department of Rheumatology, Ankara/Turkey. FAU - Kayacan, Oya AU - Kayacan O AD - Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey. FAU - Dincer, Irem AU - Dincer I AD - Department of Cardiology, Ankara/Turkey. FAU - Sayin, Tamer AU - Sayin T AD - Department of Cardiology, Ankara/Turkey. FAU - Karnak, Demet AU - Karnak D AD - Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey. Electronic address: demet.karnak@medicine.ankara.edu.tr. FAU - Turgay, Murat AU - Turgay M AD - Department of Rheumatology, Ankara/Turkey. LA - eng PT - Clinical Trial PT - Journal Article PT - Research Support, Non-U.S. Gov't DEP - 20140214 PL - Australia TA - Heart Lung Circ JT - Heart, lung & circulation JID - 100963739 SB - IM MH - Adult MH - Aged MH - Female MH - Humans MH - Hypertension, Pulmonary/*epidemiology/etiology/pathology/physiopathology/therapy MH - Male MH - Middle Aged MH - Retrospective Studies MH - Scleroderma, Systemic/complications/*epidemiology/pathology/physiopathology/therapy OTO - NOTNLM OT - Diffuse cutaneous scleroderma OT - Interstitial lung disease OT - Limited cutaneous scleroderma OT - Pulmonary arterial hypertension OT - Systemic sclerosis EDAT- 2014/03/13 06:00 MHDA- 2015/02/13 06:00 CRDT- 2014/03/12 06:00 PHST- 2013/08/27 00:00 [received] PHST- 2014/01/25 00:00 [revised] PHST- 2014/02/01 00:00 [accepted] PHST- 2014/03/12 06:00 [entrez] PHST- 2014/03/13 06:00 [pubmed] PHST- 2015/02/13 06:00 [medline] AID - S1443-9506(14)00070-5 [pii] AID - 10.1016/j.hlc.2014.02.002 [doi] PST - ppublish SO - Heart Lung Circ. 2014 Jul;23(7):667-73. doi: 10.1016/j.hlc.2014.02.002. Epub 2014 Feb 14.