Nalan Demir1, Ali Şahin2, Orhan Küçükşahin2, Oya Kayacan1, İrem Dinçer3, Tamer Sayın3, Demet Karnak4, Murat Turgay2. 1. Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey. 2. Department of Rheumatology, Ankara/Turkey. 3. Department of Cardiology, Ankara/Turkey. 4. Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey. Electronic address: demet.karnak@medicine.ankara.edu.tr.
Abstract
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. PaO₂, 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.
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. PaO₂, 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.
Authors: D Sánchez-Cano; N Ortego-Centeno; J L Callejas; V Fonollosa Plá; R Ríos-Fernández; C Tolosa-Vilella; G Espinosa-Garriga; D Colunga-Argüelles; M V Egurbide-Arberas; M Rubio-Rivas; M Freire; J J Ríos-Blanco; L Trapiella-Martínez; M Rodríguez-Carballeira; A Marín-Ballvé; X Pla-Salas; C P Simeón-Aznar Journal: Rheumatol Int Date: 2018-01-10 Impact factor: 2.631
Authors: Jacob A Macdonald; Christopher J Franҫois; Omid Forouzan; Naomi C Chesler; Oliver Wieben Journal: BMC Med Imaging Date: 2018-12-22 Impact factor: 1.930