Prevalence, risk factors and echocardiographic predictors of pulmonary hypertension in systemic lupus erythematosus: towards a screening protocol

Abstract

Background: Systemic lupus erythematosus (SLE) significantly affects the lungs and heart, and pulmonary hypertension (PH) is a severe manifestation that leads to considerable morbidity and mortality.

Objectives: We aimed to determine the prevalence and risk factors of probable SLE-PH, assess the main echocardiographic predictors and develop a potential screening strategy.

Methods: A prospective single-centre study was conducted on 201 patients with SLE who underwent transthoracic echocardiography. Patients meeting PH criteria were referred for right heart catheterisation (RHC).

Results: Among patients, 88.56% were women, 85.57% were of Spanish origin and 43.78% had structural heart disease. Out of these, 16 (7.96%) had intermediate or high probability criteria for PH according to European Society of Cardiology (ESC) 2022. Six RHCs confirmed PH with a prevalence of 2.99% for SLE-PH and 1.99% for SLE-pulmonary arterial hypertension (PAH).

Key risk factors: Key risk factors included age, cardiorespiratory symptoms, serositis, anti-Ro, cardiac biomarkers and altered pulmonary function tests (PFTs). PH was linked to a higher Systemic Lupus International Collaborative Clinics/American College of Rheumatology Damage Index (SDI) (mean SDI 4.75 vs 2.05, p<0.001) and increased mortality risk in a 2-year follow-up (12.50% vs 1.08%, p=0.002).

Conclusion: In our cohort, 7.96% of patients with SLE had an intermediate or high PH probability. By RHC, six patients (2.99%) met the ESC/European Respiratory Society criteria for PH and four (1.99%) for PAH. The main risk factors were older age, cardiorespiratory symptoms, serositis, anti-Ro, cardiac biomarkers and altered PFTs. PH was a severe SLE complication, suggesting the need for earlier diagnosis through data-driven screening to reduce associated morbidity and mortality.

Keywords: cardiovascular diseases; hypertension; lupus erythematosus, systemic; risk factors; ultrasonography.

Figure 1

Inclusion criteria flow chart. ACR/EULAR, American College of Rheumatology/European Alliance of Associations for Rheumatology; SLE, systemic lupus erythematosus; TTE, transthoracic echocardiography.

Figure 2

Flow chart of SLE-PH diagnosis. CpcPH, combined pre-capillary and post-capillary PH; IpcPH, isolated post-capillary PH; PH, pulmonary hypertension; RHC, right heart catheterisation; SLE, systemic lupus erythematosus; TTE, transthoracic echocardiography.

Figure 3

SLE-PH screening proposal. NT-proBNP, N-terminal pro-brain natriuretic peptide; PFTs, pulmonary function tests; PH, pulmonary hypertension; RHC, right heart catheterisation; SLE, systemic lupus erythematosus; sPAP, systolic pulmonary arterial pressure; TAPSE, tricuspid annular plane systolic excursion; TRV, tricuspid regurgitation velocity; TTE, transthoracic echocardiography; usTnI, ultrasensitive troponin I.