To Improve Outcomes, Identify Predictive Factors of PAH Earlier in Patients With SLE, Study Says

Although pulmonary arterial hypertension (PAH) is a rare occurrence in systemic lupus erythematosus (SLE), a recent report details efforts by researchers to find an easy way to identify predictive factors, since having both diseases together reduces overall survival.

A recent study described how investigators determined predictive factors for development of pulmonary arterial hypertension (PAH) in patients with systemic lupus erythematosus (SLE).

Although previous work has shown that the 5-year survival rate in patients with SLE in China is 92.6% in females and 81.6% in males, that percentage drops to 68% when PAH is added to the mix. However, PAH diagnosis is typically delayed by about 5 years after the initial SLE diagnosis. That delay gives more time for organ damage and worse outcomes.

Writing in the International Journal of Rheumatic Diseases, authors explained the need to recognize patients with SLE at high risk of PAH for early treatment and monitoring of their pulmonary arterial systolic pressure (PASP).

PASP is typically conducted by echocardiography. In the study, the authors conducted a chart review study in an effort to identify predictive factors in patients with a first diagnosis of SLE who were admitted to a hospital in China between January 1, 2013, and December 31, 2019.

The records of 408 patients who were first diagnosed with SLE were examined. In addition, the investigators examined demographic characteristics, clinical symptoms, autoantibodies, and laboratory tests.

Of these, 34 patients were diagnosed as having PAH by echocardiography, defined as 2 consecutive systolic pulmonary arterial pressure (PAP) values of at least 40 mm Hg.

The remaining 374 patients whose PAP value was lower than 40 mm Hg were diagnosed as having SLE without PAH.

In this study population, the prevalence of PAH in SLE was 8.3%.

Patients who had both diseases also had more occurrences of interstitial pneumonitis, polyserositis, and myocardial damage (P = .001, P = .033, and P < .001, respectively).

In addition, C-reactive protein and immunoglobin G (IgG) were higher in this group (P = .009 and P = .005).

However, the occurrence of anti–double-stranded DNA and anti–ribosomal RNA protein (anti-rRNP) antibodies were lower, indicating a lower risk of PAH (P = .004 and P = .034).

Ejection fraction and SLE disease activity index between the 2 patients groups did not differ. There were also no differences in the occurrences of Raynaud syndrome, renal involvement, arthritis, and central nervous system involvement.

The main limitations of the study were the identification of patients with PAH by echocardiography and the lack of accurate assessment of heart function, the authors said.


Pan Y, Sun Y, He L. Predictive factors for concomitant pulmonary arterial hypertension at diagnosis of systemic lupus erythematosus in a Chinese population. Int J Rheum Dis. Published online November 18, 2021. doi:10.1111/1756-185X.14251