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Severe pulmonary arterial hypertension and cardiogenic shock in acute systemic lupus erythematosus.

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January 2025

Department of Allergy, Immunology and Respiratory Medicine, Alfred Hospital, Melbourne, Victoria, Australia.

We describe a woman in her late 20s with newly diagnosed systemic lupus erythematosus (SLE), who presented with fulminant pulmonary arterial hypertension (PAH) requiring inotropic and extracorporeal support. She was established on triple pulmonary vasodilator therapy with concurrent aggressive immunosuppression; however, treatment was complicated by infection and diffuse alveolar haemorrhage, necessitating delays in immunosuppression and withdrawal of epoprostenol. Despite this, with ongoing suppression of her SLE, her pulmonary haemodynamics improved, with normal pressures on right heart catheterisation several months later allowing stepdown to sildenafil monotherapy.

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Fulminant myocarditis (FM) is an acute, diffuse inflammatory myocardial disease characterized by abrupt onset and extremely rapid progression. Patients typically exhibit haemodynamic abnormalities that may lead to respiratory failure, liver and renal failure, and subsequent coagulopathy. Collectively, these complications significantly increase the risk of early mortality.

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