The patient, a 30 year-old Caucasian female with a 6-year history of systemic lupus erythematosus was suspected of having pulmonary hypertension following chest X-Ray and routine echocardiography. Whilst awaiting further cardiological investigations she developed acute respiratory distress accompanied by gross signs of cor pulmonale and died despite full intropic and ventilatory support, in addition to intravenous "Pulse" methyl prednisolone. Postmortem findings showed typical features of the adult respiratory distress syndrome ('ARDS') but minimal vascular changes attributable to the pulmonary hypertension. The 'ARDS' was presumably associated with an acute 'flare' of the lupus.