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

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. Though long-term outcomes in patients with SLE-related PAH (SLE-PAH) remain unclear, this case highlights the importance of immunosuppression in the treatment of this cohort, with the potential for weaning pulmonary vasodilator therapy, provided that adequate control of the underlying autoimmune process is maintained.