medwireNews: Patients with pulmonary arterial hypertension (PAH) arising from systemic lupus erythematosus (SLE) may benefit from combined immunosuppressive and vasodilator therapy, a small study suggests.
Liza Rajasekhar (Nizam’s Institute of Medical Sciences, Hyderabad, India) and study co-authors found that patients who received immunosuppressive therapy in addition to a vasodilator achieved improvements in pulmonary arterial systolic pressure (PASP) that were greater than would be expected with vasodilator treatment alone.
The 24 patients in the study were all female and aged an average of 25 years. Thirteen were in New York Heart Association functional classes III or IV, six were in classes I or II and five were asymptomatic.
All patients received treatment with intravenous cyclophosphamide plus steroids, and all but four patients also received a vasodilator. After 6 months of treatment, PASP had improved in all except three patients, and 11 were classed as responders, achieving a greater than 15 mmHg fall in PASP in addition to an improved functional class.
Overall, PASP decreased significantly from 59.33 to 43.29 mmHg, which the researchers say is “higher than the expected change in PASP of 2–11 mmHg due to vasodilators alone as studied in patients with idiopathic PAH.”
Even the four patients who received immunosuppression alone, without vasodilators, had a decrease in PASP from 39.75 to 34.25 mmHg, and asymptomatic patients also experienced a reduction, from 46.4 to 34.0 mmHg.
“It is possible that immunosuppression might have added to the effect of the vasodilators”, writes the team in Rheumatology.
In total, 22 patients had an improvement in functional class, including all patients initially in classes III and IV, and the 6-minute walking distance improved in the six patients tested, from 312.04 to 420.16 m after 6 months.
Two patients died during follow-up lasting a median of 12 months, one from PAH and one due to sepsis. Neither patient had responded to treatment.
“Further studies that use catheter-based assessment of the change in PASP with treatment and randomize SLE patients to immunosuppression with vasodilators and vasodilators alone will improve our understanding of optimal therapy in this group”, the researchers conclude.
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