title: Pulmonary arterial hypertension and systemic sclerosis relation: a single centre experience.
abstract: OBJECTIVE
  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).
  
  
  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.
  
  
  CONCLUSIONS
  PAH was observed in approximately one fourth of patients; therefore advanced cardio-pulmonary investigation should be routinely performed in the SSc patients' management.
authors.affiliation: Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey.
authors.forename: Nalan
authors.initials: N
authors.lastname: Demir
authors.affiliation: Department of Rheumatology, Ankara/Turkey.
authors.forename: Ali
authors.initials: A
authors.lastname: Şahin
authors.affiliation: Department of Rheumatology, Ankara/Turkey.
authors.forename: Orhan
authors.initials: O
authors.lastname: Küçükşahin
authors.affiliation: Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey.
authors.forename: Oya
authors.initials: O
authors.lastname: Kayacan
authors.affiliation: Department of Cardiology, Ankara/Turkey.
authors.forename: İrem
authors.initials: İ
authors.lastname: Dinçer
authors.affiliation: Department of Cardiology, Ankara/Turkey.
authors.forename: Tamer
authors.initials: T
authors.lastname: Sayın
authors.affiliation: Ankara University School of Medicine, Department of Chest Diseases, Ankara/Turkey. Electronic address: demet.karnak@medicine.ankara.edu.tr.
authors.forename: Demet
authors.initials: D
authors.lastname: Karnak
authors.affiliation: Department of Rheumatology, Ankara/Turkey.
authors.forename: Murat
authors.initials: M
authors.lastname: Turgay
mesh_terms: D000328:Adult; D000368:Aged; D005260:Female; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology* / Q000209:etiology / Q000473:pathology / Q000503:physiopathology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D012595:Scleroderma, Systemic / Q000150:complications / Q000453:epidemiology* / Q000473:pathology / Q000503:physiopathology / Q000628:therapy
keywords: Diffuse cutaneous scleroderma; Interstitial lung disease; Limited cutaneous scleroderma; Pulmonary arterial hypertension; Systemic sclerosis