Pulmonary Arterial Hypertension and Systemic Sclerosis Relation: A Single Centre Experience

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• 作者：Nalan Demir ; MD^a ; Ali 艦ahin ; MD^b ; Orhan Kü ; &ccedil ; ü ; k艧ahin ; MD^b ; Oya Kayacan ; MD^a ; 陌rem Din&ccedil ; er ; MD^c ; Tamer Say谋n ; MD^c ; Demet Karnak ; MD ; FCCP^a ; ^{demet.karnak@medicine.ankara.edu.tr" class="auth_mail} ; ^{demet.karnak@gmail.com" class="auth_mail} ; Murat Turgay ; MD^b
• 关键词：Systemic sclerosis ; Diffuse cutaneous scleroderma ; Limited cutaneous scleroderma ; Pulmonary arterial hypertension ; Interstitial lung disease
• 刊名：Heart, Lung and Circulation
• 出版年：July 2014
• 年：2014
• 卷：23
• 期：7
• 页码：667-673
• 全文大小：557 K

文摘

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 (DL_CO), 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 DL_CO 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.