A Cross Sectional Study of Cardiopulmonary Complications and Severity of Pulmonary Hypertension and Lung Fibrosis in Patients With Systemic Sclerosis

摘要

Objectives: This study aims to identify the characteristics of scleroderma patients in terms of sociodemographic and clinical characteristics andseverity of lung fibrosis and pulmonary hypertension and to show the association between European Scleroderma Study Group (EScSG) scoring andseverity of pulmonary hypertension and lung fibrosis.Patients and methods: This two center cross-sectional study included 43 scleroderma patients (4 males, 39 females; mean age 52 years; range 42 to59 years) under clinical follow-up. Patients’ sociodemographic data were recorded. Median duration of illness was 10.0 years. All patients underwentblood tests, echocardiogram, full lung function test, six-minute walking test, and high resolution computed tomography of the thorax. Pulmonaryhypertension was defined by echocardiogram as systolic pulmonary pressure >36 mmHg.Results: Limited systemic sclerosis was the predominant type (74.7%). Majority of high resolution computed tomography findings showed lungfibrosis with ground glass opacities (74.4%). Pulmonary hypertension was predominantly mild (31.7%), lung function test showed 36.1% restrictivelung disease, and a reduced diffusing capacity of the lungs for carbon monoxide (median 13.85) with reduced six-minute walking test distance(median 300.0 meters). Health Assessment Questionnaire scoring was predominantly in the mild to moderate disability group (median score of 0.5).Five patients (13.9%) had active disease based on the EScSG scoring. There were no significant associations between EScSG scoring and other clinicalparameters.Conclusion: In our study, we found that scleroderma was predominant among females and the major type was limited systemic sclerosis. The lungfunction test revealed a sub-normal diffusing carbon monoxide with a reduced six-minute walking distance.