Background/Purpose: Systemic lupus erythematosus (SLE) may present hemodynamic alterations including pulmonary hypertension (PH). Echocardiography (ECHO) is a noninvasive imaging technique useful to classify pulmonary hypertension (PH) as improbable (sPAP ≤36 mmHg), possible (37-50 mmHg), or probable (>50 mmHg).

Methods: Study cohort with 6-year follow-up including 139 SLE patients. At baseline, demographics, organ involvement, disease activity (SLEDAI-2K), cumulated organ damage (SLICC/ACR), and laboratory analyses including antibody profile were obtained. Serum samples were stored in standard conditions. Clinical follow-up was performed ~3 months. SLE patients who had an ECHO for any reason by the time of recruitment (±6 months) were included. From stored sera, levels of endothelin 1 (ET1), monocyte chemotactic protein 1 (MCP1), and interferon gamma (IFNg) were measured by ELISA. Discrete variables are described as percentages and continuous variables as medians (interquartile range). Analyses were performed by either chi-square test for trends or Kruskal-Wallis with Dunn’s post-test. Spearman’s rank correlation coefficient (r) was used to assess associations. Survival was assessed by the Kaplan-Meier method with log-rank test for trends.

Results: 55 SLE patients were included. Patients were classified in 3 groups by ECHO: improbable PH (n=26, 96% female, age 34.5, 24-46 years); possible (n=16, 81% female, age 49, 38-53 years); probable (n=13, 100% female, age 41, 38-56 years). No differences in demographic or serologic features between groups, whereas organ damage was higher in patients with PH (SLICC/ACR index of 1 (1-2), 3 (1.7-4), and 3 (2-6), respectively; P=0.0009). Active arthritis was present in 12%, 13%, and 38% (P=0.05); history of pulmonary thromboembolism in 8%, 13%, and 46% (P=0.005). Serum levels of ET1, MCP1 and IFNg were similar between groups. The sPAP showed a positive correlation with age (ρ 0.29), disease duration (ρ 0.32), serum creatinine (ρ 0.26), SLEDAI-2K (ρ 0.26), SLICC/ACR (ρ 0.55), left atrium diameter (ρ 0.45), interventricular septum thickness (ρ 0.33), and right ventricular diastolic diameter (ρ 0.71). An inverse correlation with C3 (ρ -0.25) and CH50% (ρ -0.25) was found. Main causes of PH (sPAP >37) were: connective tissue disease (32%), intrinsic cardiac disease (26%), pulmonary thromboembolism (26%), and pulmonary disease (16%). Rates of survival in the first year were: improbable PH 92%, possible PH 94% and probable PH 90%. After three years, these were 92%, 89% and 77%, respectively. After six years 88%, 87% and 68%, respectively.

Conclusion: In SLE, PH (sPAP >50 mmHg) is associated with decreased survival in the medium term. Also, it is related to cumulated organ damage and history of pulmonary thromboembolism. Validated biomarkers in idiopathic PH such as ET1, MCP1 and INFg as well as SLE- and scleroderma-related autoantibodies are not useful to distinguish PH in patients with SLE.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/pulmonary-hypertension-in-systemic-lupus-erythematosus-a-6-year-follow-up-study-cohort/