When the immune system strikes twice: SLE-associated pulmonary arterial hypertension: a case report

Both SLE-PAH flares followed discontinuation of maintenance immunosuppressive therapy, underscoring that continuous immunosuppression might reduce flare risk. The clinical course during flares aligns with evidence that SLE-associated PAH benefits from immunosuppression, in contrast to other PAH forms. Finally, the case highlights that negative chronotropic-inotropic agents (e.g. β-blockers) can induce life-threatening haemodynamic shock in patients with PAH.