Reversibility of pulmonary hypertension in systemic lupus erythematosus after induction immunosuppressive therapy: An inflammatory manifestation?

Objective To evaluate the possible reversibility of PAH to a normopressoric state in SLE after induction immunosuppressive (IS) and predictors of response. Methods We retrospectively evaluated all SLE-PAH patients who underwent IS therapy at our center. PAH reversion was defined as the normalization of pulmonary arterial pressure (PAP), either by the presence of systolic PAP <40 mmHg on echocardiogram or mean PAP <20 mmHg on right heart catheterization (RHC). SLE patients were divided in Reversion and No-Reversion of SLE-PAH groups for comparative analysis at baseline and after IS. Results Among 2,074 SLE patients, 28 SLE-PAH received IS therapy (1.3%). Ten patients (35.7%) achieved SLE-PAH reversion. Demographic data, disease duration, SLEDAI-2K, and SDI Damage scores were similar between Reversion and No-Reversion of SLE-PAH groups ( p > 0.05). At baseline, Reversion of SLE-PAH had lower sPAP ( p = 0.032), lower right ventricle dilatation ( p = 0.003) and hypokinesia ( p = 0.017) frequencies on echocardiogram, and also lower BNP levels ( p = 0.041) and risk stratification score ( p = 0.014). Hemodynamic parameters were similar among groups ( p > 0.05). After IS, a significant decrease in CRP levels was identified only in Reversion of SLE-PAH ( p = 0.013), although both groups had a significant reduction in SLEDAI-2K ( p < 0.05). Both groups had significant improvement in risk stratification score ( p = 0.009 and p < 0.001) with a better survival rate in Reversion of SLE-PAH ( p = 0.047). Conclusion This is the first study that identified that more than one third of SLE-PAH had a complete reversion of PAH after IS therapy with a significant impact on their survival. These findings strongly support the notion of an underlying inflammatory etiology of this condition, which reinforces the use of immunosuppressive treatment for all SLE patients at PAH onset.