Background and aims Immune and inflammatory mechanisms could play a significant role in pulmonary arterial hypertension (PAH) genesis and progression, especially in patients with systemic lupus erythematosus (SLE). Immunosuppressive therapy should be better evaluated in this setting. We reviewed the clinical outcomes of intensive immunosuppressive therapy with or without target therapy in SLE associated P
Methods This single-centre cohort study enrolled 126 consecutive patients with SLE-PAH who visited our referral centre in China between May 2006 and December 2015. Baseline demographics, clinical features, laboratory results, haemodynamic assessments and management were analysed. Kaplan-Meier curves and Cox proportional hazards regression analysis were used toevaluate the role of intensive immunosuppressive therapy.
Results ALL patients received intensive immunosuppressive therapy including combination of high-dose glucocorticosteroids and first-line immunosuppressants, such as cyclophosphamide, mycophenolate and calcineurin Inhibitors. Eighty-two (65.1%) patients received target therapy at baseline. Survival analysis indicated that responders had a better survival than nonresponders in both with and without target therapy group (Figure 1). Patients with a shorter SLE disease duration (p=0.009) and better baseline pulmonary hemodynamics (mean pulmonary arterial pressure, pulmonary vascular resistance and Cardiac index, p<0.001) were more likely to benefit from immunosuppressive therapy (Table 1).
Conclusions Intensive immunosuppressive therapy markedly improved the long-term outcomes of SLE patients with PAH, especially in the early stage of PAH.
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