Chest
Clinical Investigations: Pulmonary VasculatureClinical Evaluation of Pulmonary Hypertension in Systemic Sclerosis and Related Disorders: A Doppler Echocardiographic Study of 135 Japanese Patients
Section snippets
Patient Selection and Study Protocol
The initial study population consisted of 154 Japanese patients who were evaluated at the Scleroderma Clinic of Tokyo University Hospital, and who underwent multiple radiographic, echocardiographic, and pulmonary function studies between March 1990 and December 1995. Inclusion in the study required the presence of sclerodermatous skin changes or puffy, sausage-like fingers,18, 19 and a positive immunofluorescence test result for antinuclear antibodies.16, 17 We excluded 14 patients with
Characteristics of the Study Patients
Table 1 shows the characteristics of the final study population, which consisted of 135 patients (122 women and 13 men; mean age, 52.7±12.3 years). The mean period from the disease onset to the last echocardiographic study (duration of disease) was 11.6±8.7 years. Thirty-three patients with distal scleroderma did not meet the ARA diagnostic criteria for systemic sclerosis.20 These patients were considered to have systemic sclerosis or related disorders on the basis of the presence of
DISCUSSION
In this study of Japanese patients with systemic sclerosis and related connective tissue disorders, Doppler-estimated pulmonary artery pressures were significantly higher in the proximal scleroderma and in the SLE/PM overlap groups. The FVC and Dco were significantly reduced in the proximal scleroderma than in the distal scleroderma group, suggesting that pulmonary fibrosis was the primary cause of PH in the proximal scleroderma group. Pulmonary arteriopathy was the probable cause of PH in the
ACKNOWLEDGMENTS
The authors are indebted to Susumu Sakurai and Tomiko Takahashi for their fine technical assistance during the echocardiographic studies. We also thank Dr. Chikuma Hamada, of the Department of Pharmacoepidemiology, for his kind advice regarding the statistical analyses.
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Determinants of Pulmonary Arterial Hypertension in Scleroderma
2007, Seminars in Arthritis and RheumatismCitation Excerpt :The prevalence (29%) of PAH found in this study is within the range reported by previous cross-sectional studies (4,5,16-22). In the majority of these studies, screening for PAH was similarly done by 2-dimensional echocardiography, with the RVSP cutoff values varying from 30 to 45 mm Hg (4,15,16,18,23). Studies (4,6,15,16,18,22,24) employing right heart catheterization for the diagnosis of PAH have reported lower prevalence rates compared with studies using echocardiography (ranging from 5 to 12% versus 13 to 35%, respectively).
Right ventricle Tei-index: A tool to increase the accuracy of non-invasive detection of pulmonary arterial hypertension in connective tissue diseases
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Supported in part by a research grant to study mixed connective tissue disease from the Ministry of Health and Welfare of Japan.