To evaluate the prevalence of Pulmonary Hypertension in unselected patients with Systemic Lupus Erythematosus (SLE).
This is a cross sectional study in a tertiary care centre. 50 consecutive, unselected SLE patients were enrolled and had a transthoracic echocardiogram to look for evidence of Pulmonary Hypertension (PH), including Right Ventricular Systolic Pressure (RVSP), RA size, Right Ventricular (RV) systolic dysfunction, pericardial effusion, Tei index, Tricuspid Annular Plane Systolic Excursion (TAPSE). PH was defined as tricuspid regurgitation jet velocity > 2.7m/sec or RVSP > 35 mmHg. Patients with any reported dyspnea (n = 2) and fourteen other randomly selected subjects (clinical cohort, n = 16) had further evaluation. This included clinical assessment, chest X-ray (CXR), full Pulmonary Function Tests (PFT), and 6 Minute Walk Test (6MWT). High Resolution CT (HRCT) chest was done if there was any suspicion of possible Interstitial Lung Diseases (ILD) based on either clinical evaluation, CXR, or on physiologic testing.
50 subjects (age 47 ± 12 [mean ± SD], 49 female [98%]) with SLE (duration 11 ± 9 years, ARA criteria 4.7 ± 1) were evaluated with echocardiogram. None was found to have any echocardiographic findings suggestive of PH. In the clinical cohort of n = 16, there were no physical examination evidence of PH. Hemoglobin-corrected Diffusion Capacity (DLco) was 72 ± 14 %predicted, with 7 subjects having DLco < 75% predicted. Of these, 3 had Total Lung Capacity (TLC) < 80% or abnormal HRCT with evidence of ILD or emphysema. 4 subjects had reduced DLco in the absence of any symptoms, PFT or pulmonary imaging abnormalities.
In 50 unselected patients with SLE, we identified no cases of PH based on clinical symptoms, physical exam, or echocardiographic findings. However, 4 subjects had unexplained, isolated reduced DLco, suggesting 8% (95% CI 0–16%) of unselected SLE could have early, subclinical, asymptomatic, echocardiographically-negative pulmonary vascular disease.
Although PH is less common in patients with SLE than other types of CTD, such as scleroderma, unexplained isolated reduction in DLco occurs in 8%, and could be an early indicator of PH, and thus probably merits clinical follow up.
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