Pulmonary hypertension in systemic lupus erythematosus

Abstract

A prospective echocardiographic and clinical study was performed on 84 Chinese patients with systemic lupus erythematosus (SLE) and 99 controls to investigate the prevalence and the mechanism of pulmonary hypertension (PH) in SLE. Comparison between Doppler estimation and catheterization measurement was made in 12 cases to validate the predictive method. Compared to normal subjects, lupus patients had significantly increased systolic pulmonary artery pressure (SPAP) (29.59 +/- 12.52 vs 19.64 +/- 5.82, P < 0.001), mean pulmonary artery pressure (MPAP) (15.11 +/- 7.36 vs 10.21 +/- 4.72, P < 0.001) and total pulmonary resistance (TPR) (315.85 +/- 190.65 vs 220.37 +/- 55.92, P < 0.001). Nine of the 84 patients presented PH, defined as SPAP < 30 mmHg and MPAP > 20 mmHg. Pulmonary hypertensive patients had higher serum endothelin (ET) than non-pulmonary hypertensive patients, were more commonly in active stages, and presented Raynaud's phenomenon and rheumatoid factors. ET level was correlated with echocardiographic pulmonary pressure. Pulmonary hypertension commonly occurs in Chinese patients with SLE (11%), and it correlates with the lupus activity and the elevation of serum endothelin.

Disease Activity Is Related to Acute Response to Vasodilator in Pulmonary Artery Hypertension Associated With Systemic Lupus Erythematosus

“…17-19 Some studies have suggested an imbalance between vasoconstrictors and vasodilators in SLE-APAH. 20 According to the present results, there might be difference in the principal mechanism of SLE-APAH according to SLE activity. In the present study, at a similar level of mPAP, lower SLEDAI was related to higher PVR, larger RV size, and worse RV longitudinal systolic function, which might indicate severer PAH in these patients.…”

Pulmonary arterial hypertension related to connective tissue diseases

“…Bir araştırmaya göre PAH saptanan 105 SLE hastanın %90'ı kadın, ortalama yaş 33, PAH tanısından sonraki 2 yılda ölüm oranı %25-50 arasındadır (34). Çinlilerin yaptığı 84 hastalık çalışmada ekokardiyografi ile PAH %11 oranında gözlenmiştir, ancak SKK ile doğrulama yapılmamıştır (35). Ekokardiyografi sadece tarama amaçlı kullanılır, yüksek yalancı pozitiflik görülebilir.…”

Scleroderma and pulmonary hypertension

“…interstitial lung disease) (9) . Pulmonary hypertension has been reported in 4%-14% of the patients with systemic lupus erythematosus (SLE) with an overall mortality rate of 25%-50% at two years from diagnosis of pulmonary hypertension (19)(20)(21)(22)(23)(24)(25) . Patients with mixed connective tissue disease (MCTD) have features of several connective tissue diseases including SLE, scleroderma, rheumatoid arthritis and polymyositis (26,27) .…”