Outpatient Raynaud Treatment Saves Fingers and Toes

When Monique Hinchcliff, MD, MS, associate professor of medicine (rheumatology, allergy & immunology) and director of the Yale Scleroderma Program, first came to Yale, the only way to care for patients with severe, treatment-resistant Raynaud phenomenon—whose fingers were turning black—was to send them to the emergency department for hospitalization and treatment.

The system wasn’t ideal. “We wanted to be able to help these patients receive the care they needed in a relaxed, non-urgent setting, in a way that could be planned ahead of time,” Hinchcliff says.

Raynaud is a condition that involves decreased blood flow to certain body parts, especially fingers and toes. Common triggers of the disorder include cold exposure and stress. Raynaud phenomenon—in which the disorder is associated with underlying conditions such as scleroderma or other autoimmune diseases—can be severe, causing tissue damage and even finger loss.

“Patients with scleroderma get tiny vessel disease that can lead to painful ulcers and black fingertips that, if not treated, can cause tremendous disability and, in the most serious cases, result in amputation,” Hinchcliff says. “But the medication epoprostenol, administered intravenously, can prevent or treat these ulcers and gangrene by relaxing blood vessels, improving circulation and healing blood vessels.”

The treatment is used as a last resort in patients who have autoimmune disease and acute circulatory problems that have not responded to standard medical therapy, explains Vaidehi Chowdhary, MD, associate professor of medicine and clinical chief (rheumatology, allergy & immunology).

To avoid sending patients to the emergency department, Hinchcliff developed a process to have them treated in the medical intensive care unit (ICU). But during the COVID-19 pandemic, the ICU beds were needed for patients with the virus. So Hinchcliff set out to find a place where epoprostenol could be given intravenously in an outpatient setting.

There were challenges along the way, Hinchcliff recalls. The medication is approved by the U.S. Food and Drug Administration (FDA) to treat patients with pulmonary arterial hypertension at much higher doses that come with side effects. Hinchcliff had to educate other physicians about the much smaller doses—and lower risk—involved in treating Raynaud phenomenon. In addition, administering epoprostenol requires specially ordered pumps that staff would need training to learn how to use.

After developing an EPIC order, creating a sequence of steps, and conducting educational sessions about the treatment, Hinchcliff’s efforts paid off. Epoprostenol is now given to patients in infusion suites at Yale New Haven Hospital, Saint Raphael Campus, Yale New Haven Health Fairfield, Yale New Haven Health North Haven, Greenwich Hospital, and Yale Health.

Hinchcliff sees Yale as a model for how epoprostenol can be offered intravenously in an outpatient setting in the US. “Physicians at other organizations also have requested access to the protocol, which we readily share. We’re glad to help support efforts to make this treatment available to more patients in additional outpatient settings throughout the country,” says Hinchcliff.

Chowdhary says they hope to continue rolling out the treatment to other infusion centers. “We want to continue to improve access for patients who are in remote parts of Connecticut, especially during the winter, when Raynaud becomes a big problem,” she says. “We hope to expand this program to include all YNHHS centers.”

Both Hinchcliff and Chowdhary emphasize the amount of collaboration that went into establishing the new process. It was a big ask of the nurses, pharmacists, physicians, and schedulers, Chowdhary says, but they rose to the challenge.

“This was a collaborative effort that required all stakeholders to get involved, raise concerns, and work together to address any challenges,” says Hinchcliff. “

Now that the outpatient treatment is in place, Yale researchers are assessing its effectiveness by administering a newly developed patient questionnaire specifically for Raynaud phenomenon and using a handheld digital camera to capture images of patients’ finger nailfold capillaries. Patients also undergo hyperspectral imaging—through a special camera lent to Hinchcliff and her team by Bauer Sumpio, MD, PhD, professor of surgery (vascular) and of radiology and biomedical imaging and of medicine (cardiology)—that shows the amount of oxygen in the fingers.

The goal, Hinchcliff says, is to identify which patients benefit most from epoprostenol and to determine the optimal duration of treatment, including how many times to administer infusions each month and for how many months. Also unanswered, Hinchcliff says, is whether, once the treatment is successful, it will need to be repeated after a certain amount of time.

Many patients have shared positive feedback about their experience, Hinchliff says. “Some of our patients experienced dramatic improvement in digital perfusion. Others noticed less improvement, but—importantly—the treatment was well-tolerated, and there have not been any serious adverse events.”

Editor’s Note: This article was originally published in August 2023 and was updated to reflect new developments and insights.

Rheumatology, Allergy & Immunology, one of 10 sections in the Yale Department of Internal Medicine, is dedicated to providing care for patients with rheumatic, allergic and immunologic disorders; educating future generations of thought leaders in the field; and researching fundamental questions of autoimmunity and immunology. To learn more, visit Rheumatology, Allergy & Immunology.