Clinical Characteristics of Scleroderma Patients With and Without Pulmonary Hypertension

Abstract

PURPOSE: Scleroderma is a connective tissue disorder which is often complicated with pulmonary hypertension (PH). The aim of the study was to investigate potential differences in the clinical characteristics of scleroderma patients with and without PH, estimated by resting Doppler echocardiography. METHOD(S): A consecutive population of 82 scleroderma patients (11.9% male; 49.8 years old and 88.1% female; 54.9 years old), who were evaluated at the respiratory physiology laboratory of the Respiratory Failure Unit, constituted the study population. Patients underwent spirometry, diffusion capacity measurement and maximum cardiopulmonary exercise testing on a cycle ergometer. Data on anthropometric characteristics, thorax computed tomography imaging and blood serology were also recorded. RESULT(S): Twenty eight patients (34.15%) presented with PH (systolic pulmonary arterial pressure >35 mmHg). Patients with PH had also lower FEV1 %predicted (p<0.001), FVC %predicted (p<0.001), IC %predicted (p=0.041), TLC %predicted (p=0.032), FRC %predicted (p<0.001), and DLCO %predicted (p=0.01) and exhibited more often pulmonary fibrosis in CT scan, versus those without PH. As expected, those with PH had lower VO2 max%predicted compared to the ones without PH (p=0.035), while no difference was noted in the autoantibodies subtype (antisclero70 or ACA) between the two groups. CONCLUSION(S): Although two different types of scleroderma patients (one with pulmonary fibrosis and one with pulmonary hypertension) have been described, in the current study patients with pulmonary vasculopathy also presented with worse respiratory function.