Abstract Background/Aims Pulmonary arterial hypertension (PAH) is a rare but severe complication of connective tissue disease (CTD), including systemic lupus erythematosus (SLE). Immune-mediated pulmonary vascular remodelling and endothelial dysfunction drive rapid haemodynamic deterioration and high mortality. Current guidelines recommend parenteral prostacyclin therapy, such as intravenous epoprostenol, for high-risk PAH (WHO Functional Class IV). In contrast to idiopathic disease, PAH associated with inflammatory CTD, such as SLE, may demonstrate reversibility with timely immunosuppression. Methods A 35-year-old lady with active SLE on long-term hydroxychloroquine, diagnosed in 2017 on the basis of positive anti-dsDNA antibodies, low complement C3/C4, positive antiphospholipid antibodies, autoimmune haemolytic anaemia, and thrombocytopenia, presented with progressive exertional dyspnoea, presyncope, and peripheral oedema. Echocardiography demonstrated right ventricular dilatation and dysfunction, elevated pulmonary artery pressures and pericardial effusion. Before urgent transfer to the national pulmonary hypertension centre for further evaluation, she was commenced on prednisolone 1mg/kg for active lupus and autoimmune thrombocytopenia. Right heart catheterisation (RHC) confirmed pre-capillary pulmonary hypertension with mean pulmonary artery pressure (mPAP) of 66mmHg, consistent with severe pulmonary hypertension, while CT lungs and ventilation-perfusion scanning excluded alternative causes. According to ESC/ERS 2022 guidelines, she was classified as high risk, warranting upfront triple combination therapy including intravenous epoprostenol. However, severe thrombocytopenia (platelets 19 × 109/L, normal: 150-400) made prostacyclin therapy unsafe due to bleeding risk. A multidisciplinary team involving rheumatology, haematology, and pulmonary hypertension specialists determined that bleeding risk outweighed potential haemodynamic benefit at the time. Intensive immunosuppression with corticosteroids and rituximab was therefore commenced, aiming to improve thrombocytopenia. Oral dual pulmonary vasodilator therapy with a phosphodiesterase-5 inhibitor (tadalafil) and an endothelin receptor antagonist (macitentan) was initiated cautiously, resulting in clinical stabilisation. Results Over two weeks, NT-proBNP levels, symptoms, and exercise tolerance improved. Platelets improved to 124 × 109/L. Repeat echocardiography showed marked improvement in right ventricular size and function with reduced systolic pressures; RHC showed mPAP of 37mmHg (moderate PAH). Her risk status improved from high to intermediate risk, and a third oral vasodilator, selexipag, was subsequently introduced. Conclusion PAH is a recognised but uncommon and life-threatening manifestation of SLE. Unlike idiopathic PAH, the pathophysiology in SLE often involves immune-mediated endothelial injury and pulmonary vascular remodelling, creating a therapeutic window where early immunosuppression can improve outcomes. Current ESC/ERS guidelines recommend parenteral prostacyclin therapy for high-risk disease; however, treatment must be individualised when complications like thrombocytopenia increase bleeding risk. In this case, profound thrombocytopenia precluded standard high-intensity vasodilator therapy. A multidisciplinary approach enabled tailored management with prompt immunosuppression (corticosteroids and rituximab) alongside oral dual vasodilator therapy, leading to significant clinical and echocardiographic improvement. This highlights the importance of potential reversibility of SLE-associated PAH with timely immunosuppression and importance of multidisciplinary, individualised care in complex presentations where standard therapy poses risk. Disclosure W. Lai: None. L. Price: None. C. McCabe: None. S.J. Wort: None. M. Fernando: None. H. Nashat: None.
Lai et al. (Wed,) studied this question.
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