Intravenous Iloprost May Slow or Halt Disease Progression in Scleroderma Patients, Study Finds

Intravenous administration of iloprost may stabilize and improve symptoms of dysfunctional cardiopulmonary and blood vessel activity in patients with systemic sclerosis, possibly halting disease progression, according to new research.

The study, “Long-Term Clinical Stabilization Of Scleroderma Patients Treated With A Chronic And Intensive IV Iloprost Regimen,” was published in the journal Rheumatology International and conducted by researchers in Italy.

Iloprost – a drug that has been approved by the U.S. Food and Drug Administration (FDA) under the name Ventavis for the treatment of pulmonary arterial hypertension, a cardiopulmonary disease – can also be used in the treatment of scleroderma-associated blood vessel anomalies in the fingers. Previous studies have suggested that this drug may have a beneficial role on disease progression.

To evaluate the effect of iloprost in the progression of systemic sclerosis in terms of cardiopulmonary function, researchers analyzed 68 scleroderma patients with a mean age of 54.4 years who had been treated with intravenous iloprost for about seven years. Iloprost was administered for five to six consecutive days each month.

After the treatment, all patients showed significant improvements in several areas, including the modified Rodnan skin score (a measure of sclerosis in the skin); systolic pulmonary arterial pressure (sPAP); tricuspid annular plane systolic excursion (an estimate of systolic function); and pro-brain natriuretic peptide (high levels of this protein indicate an abnormal heart effort).

In a subgroup of patients who had an sPAP above or equal to 36 mmHg (17 patients), a significant reduction was observed (from a mean of 39.5 to 25.1 mmHg) after 7.6 years of treatment plus 2.5 years of follow-up.

Also, treatment with IV iloprost reduced the number of patients with digital ulcers (a consequence of impaired blood flow in the fingers, due to scleroderma) at follow-up from baseline (42.6 to 11.8 percent). No patient free of digit ulcers at the start of the study presented ulcers after the treatment.

Together, the results showed that continued treatment with IV iloprost may improve cardiopulmonary function and blood vessel anomalies in systemic sclerosis patients.

“Scleroderma remains a treatable but not curable disease, characterized by a poor prognosis due to the occurrence of cardiopulmonary complications,” the authors wrote in their study.

“Therefore, the long-term disease stabilization represents an important therapeutic goal. Intravenous iloprost may play a role in promoting a favorable disease course during a long-term follow-up,” they wrote. “Our results confirm that monthly iloprost infusions for six consecutive days might exert a beneficial healing effect and reduce the occurrence of new [digital ulcers] as well against cardiopulmonary disease development or worsening in [scleroderma] patients.”