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Pulmonary hypertension secondary to systemic lupus erythematosus: prolonged survival following treatment with intermittent low dose iloprost

Room 448, 4=F, Windeyer Building, 46 Cleveland Street, London W1P 6DP, UK

H F Tse

Divisions of Rheumatology and Cardiology, University Department of Medicine, Queen Mary Hospital, Hong Kong

C S Lau

Divisions of Rheumatology, University Department of Medicine, Queen Mary Hospital, Hong Kong

Pulmonary hypertension (PHT) associated with systemic lupus erythematosus (SLE) has a dismal prognosis. Vasodilators and immunosuppressive therapy have been tried over the years with discouraging results. Prostacyclin (PGI₂) which has potent vasodilatatory and anti-platelet effects has been demonstrated to significantly decrease pulmonary arterial pressure and pulmonary vascular resistance during acute infusion. Satisfactory response has been reported in SLE patients with PHT treated with short-term intravenous continuous PGI₂ infusion. We report here a 48-month experience of the use of monthly low dose infusion of a PGI₂ analogue, iloprost, in a SLE patient with pulmonary hypertension in New York Heart Association functional Class III. There was an initial haemodynamic response to an acute infusion of iloprost. Repeated infusions were followed by marked improvement in her functional status and her mean pulmonary arterial pressure dropped from 80 mmHg in the first few months and remained static at around 55 mmHg for the subsequent years.

Key Words: prostaglandin • prostacyclin • connective tissue disease • secondary pulmonary hypertension

Lupus, Vol. 8, No. 4, 328-331 (1999)
DOI: 10.1191/096120399678847795


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