5-YEAR FOLLOW-UP-STUDY OF THE PREVALENCE AND PROGRESSION OF PULMONARY-HYPERTENSION IN SYSTEMIC LUPUS-ERYTHEMATOSUS

The purpose of this study was to determine the prevalence and progress ion of pulmonary hypertension over a 5-year follow-up period in 28 pat ients with systemic lupus erythematosus (SLE) who were originally enro lled in an echocardiographic study of pulmonary hypertension in 1985 a nd 1986. Twenty healthy volunteers without cardiac or pulmonary diseas e participated as normal controls. Each patient and control underwent a complete Doppler echocardiographic study. Doppler echocardiographic recordings of tricuspid insufficiency, with saline contrast enhancemen t when necessary, were used to calculate pulmonary artery systolic pre ssure according to the modified Bernoulli equation. Doppler echocardio graphic measurement of cardiac output was performed at rest for each s ubject, and pulmonary resistance was calculated by dividing the pulmon ary artery systolic pressure by the cardiac output. These results were compared to results of the original studies to detect serial changes in pulmonary pressure and pulmonary resistance; results were also comp ared to the group of normal controls. The prevalence of pulmonary hype rtension increased from 14% at the first study to 43% at follow-up. A significant increase in mean systolic pulmonary artery pressure was de tected in the SLE patients during the follow-up period: 23.4 vs 27.5 m m Hg (p < 0.005). In addition, a significantly higher pulmonary artery pressure was detected in the SLE patients compared with the normal co ntrols (p < 0.005). An increase in pulmonary resistance during the fol low-up period was detected for the SLE group as a whole (p < 0.001) an d for the subgroup of patients with pulmonary hypertension at the seco nd study (p < 0.001), implying that the mechanism for pulmonary hypert ension was an increase in pulmonary vascular resistance. In conclusion , pulmonary hypertension is common in SLE, is gradually progressive ov er time, and is related to an increase in pulmonary resistance.