Background/Purpose:

Intravenous prostanoid therapy is recommended for severe systemic sclerosis related digital vasculopathy. Evidence to support this recommendation is limited. Our objective is to evaluate the safety and efficacy of treating scleroderma spectrum digital vasculopathy with intravenous epoprostenol.

Methods:

Patients with systemic sclerosis and received intravenous epoprostenol for scleroderma spectrum digital vasculopathy (defined as digital ischemic ulcers, digital ischemia with or without gangrene, but not uncomplicated Raynaud’s) between 10/01/2003 – 09/01/2015 at Boston University Medical Center were identified using ICD-9 code search and their charts were reviewed in this retrospective case series. The epoprostenol infusion protocol used was as follows:

Continuous intravenous epoprostenol begun at 2ng/kg/min and increased every 15min to a maximum dose of 8ng/kg/min. Epoprostenol was administered via central access (most frequently a PICC line); the final dose was dependent on tolerability, hemodynamic stability and oxygenation. This dose (most frequently 8ng/kg/min) was maintained for five days and then weaned by 2ng/kg/min every 12h unless there was recurrence of the digital ischemia. In that case, the dose was held or increased, depending on the severity of the ischemia, until the ischemic episode resolved. Prior to discontinuing the epoprostenol, another vasodilator (usually a PDE-5i) was added.

Results:

There were 46 epoprostenol infusions in 35 patients with scleroderma spectrum digital vasculopathy. 32 patients were female. Patients’ ages ranged from 21 to 85 with a median age of 50. 19 patients had limited cutaneous systemic sclerosis, 5 had diffuse cutaneous systemic sclerosis, 3 had mixed connective tissue disease, 2 had systemic sclerosis sine scleroderma. Scleroderma spectrum subtype was not documented in 6 patients. Epoprostenol was usually titrated to 8ng/kg/min and held at that dose for 5 days.

Out of 46 epoprostenol infusions, 29 had documentation of improvement as indicated by pain relief, increased perfusion of digits as assessed by warmth or color, reduced number and size of digital ulcers. Two epoprostenol infusions resulted in no improvement. In 16 infusions, there was no documentation as to whether the digital vasculopathy improved or not. Although there was no documented clinical deterioration during an infusion, 5 patients ultimately required surgical intervention including amputation or debridement.

Out of 46 infusions, adverse effects occurred in 26. Adverse events were common and included nausea/vomiting, jaw pain, headaches and flushing, but were transient and typically occurred with dose escalation. Serious adverse events were uncommon and included hypotension (4), line infection (1) and line thrombosis (1).

Conclusion:

This is the largest study investigating the use of intravenous epoprostenol infusion in the treatment of scleroderma spectrum digital vasculopathy. We demonstrate that the use of our intravenous epoprostenol infusion protocol vasculopathy is generally safe and effective, and should be considered an important therapeutic option for scleroderma spectrum digital vasculopathy.