Pulmonary Hypertension in Systemic Lupus Erythematosus

Pulmonary Thromboembolism Is the Leading Cause

Ali Akdogan, MD; Levent Kilic, MD; Ismail Dogan, MD; Sercan Okutucu, MD; Elif Er, MD; Bariş Kaya, MD; Lutfi Coplu, MD; Meral Calguneri, MD; Lale Tokgozoglu, MD; Ihsan Ertenli, MD*


J Clin Rheumatol. 2013;19(8):421-425. 

In This Article

Abstract and Introduction


Background: Pulmonary hypertension (PH) is a life-threatening complication of systemic lupus erythematosus (SLE). Pulmonary hypertension in SLE has a variety of causes. Diagnosing early and defining the cause of PH accurately can provide better clinical outcome in SLE. We investigated the causes and characteristics of PH in patients with SLE.

Methods: One hundred twenty-one patients with SLE who had a visit in a 6-month period were assessed retrospectively. Patients who ever had a systolic pulmonary arterial pressure of 40 mm Hg or greater by Doppler echocardiography were considered to have PH.

Results: Among 122 patients, 65 had echocardiography for some reason, and 10 (8.2%) were diagnosed as having PH by echocardiographic examination. This number reduced to 9 (7.4%) when we excluded the patient with normal pulmonary artery pressure at right heart catheterization. Causes of PH were as follows: thromboembolic events in 4 patients (44.4%) (2 of them had chronic thromboembolic PH), left-sided heart disease in 2 patients (22.2%), pulmonary arterial hypertension in 1 patient (11.1%), high cardiac output state in 1 patient (11.1%), and transient elevation of systolic pulmonary artery pressure in 1 patient (11.1%) who had a history of venous thromboembolism. Venous thromboembolic disease was significantly higher in patients with SLE with PH in comparison to patients with SLE without PH (7 patients [6.3%] vs 5 patients [50.0%]; P = 0.001). All patients improved clinically during their short-term follow-up.

Conclusions: Patients with SLE are at increased risk for PH. This study highlights the complexity of the differential diagnosis of PH in patients with SLE once again and emphasizes the importance of pulmonary thromboembolism as a cause of PH. One should investigate patients with SLE with unexplained symptoms and/or signs related to PH for possible treatable causes.


Pulmonary hypertension (PH) is considered to be a rare and serious complication of systemic lupus erythematosus (SLE).[1,2] Pulmonary hypertension has numerous causes and is classified in 5 main clinical groups: pulmonary arterial hypertension (PAH, group 1), PH due to left-sided heart disease (group 2), PH due to lung diseases (group 3), PH due to chronic thromboembolic PH (CTEPH, group 4), and PH with unclear and/or multifactorial mechanisms (group 5). All of these groups include conditions that can be associated with SLE.[1,2] Regardless of the etiology, the presence of PH is a poor prognostic factor.[3–6] Prevalence of PH is reported to be 9% to 14% in studies performed by echocardiography in SLE but lower in other studies based on hemodynamic parameters.[7,8] The aim of this study was to determine the causes and characteristics of PH in patients with SLE.