Our Journeys

PH Specialist Story

Virginia Steen, MD

Dr Virginia SteenMeet Dr. Virginia Steen: A Dedicated Partner in the PH Field

Virginia D. Steen, MD, is Professor of Medicine at Georgetown University Medical Center where she also serves as the fellowship director in the rheumatology division and as a consultant in rheumatology in the arthritis division of the NIH. Recently, Dr. Steen spoke with PHA about her work with scleroderma and PH patients.

You specialize in rheumatology. What sparked your interest in PH?

I have been interested in PH in scleroderma for more than 25 years. My research interest is the epidemiology of scleroderma, particularly with regard to the scleroderma database out of the University of Pittsburgh, which I helped establish. I was involved with a study that followed a group of scleroderma patients with PH. It was the 1980s and there was no treatment for PH. As treatments became available, I knew it was extremely important to identify scleroderma patients with PH as soon as they were diagnosed. After all, we had a population of patients who we knew was at risk. I continued my research to identify which patients were at greatest risk for PH and found that those with limited cutaneous scleroderma with longstanding Raynaud's disease, an anti-centromere antibody, and those with a very low DLCO (a valuable test of lung function) were at greatest risk.

What advances have you seen in the treatment of PH patients?

It has been really wonderful NOT to have to tell patients they are going to die in two years and we have nothing to treat them with. True, PH is still a serious complication in scleroderma but it is definitely treatable. It's exciting to see the number and variety of treatments grow.

Can you tell us about your experience treating scleroderma patients who have PH?

For many reasons, treating PH in scleroderma is more difficult than treating idiopathic PAH (IPAH). Patients are often older, have complicating medical problems, and perhaps other conditions. Additionally, the PH in scleroderma is often not pure PAH; our patients often have some component of interstitial lung, myocardial fibrosis, and even venoocclusive disease in addition to pulmonary arterial disease. For all these reasons, the disease is not as responsive to treatment as it is in IPAH. Scleroderma patients are not complainers, they adapt to their disability, and far too often they don't get diagnosed until they are already functional class III with right heart failure. It is critical that we identify these patients sooner and get them treated as early as possible.

From your time working in the PH field, do any cases really stand out to you?

The first PH patient I treated with an endothelin receptor antagonist was a 72-year-old woman who kept insisting she wasn't short of breath. She had significant PH, and even though she denied shortness of breath, I treated her anyway. She came back three months later and almost ran into my office hugging me and telling me she never realized how short of breath she was. She was now able to go to church (to climb the stairs to get to the church), which she hadn't been able to do for three years. I had another 72-year-old scleroderma patient who for three years had been doing very well on oral agents but kept requiring more and more oxygen. At that point she was ready to give up because she didn't want to have to deal with the more invasive medication. However, after several long discussions, she decided to try intravenous therapy, and now two years later, she rarely needs oxygen and just returned from seeing her new grandchild.

How did you become involved with PHA?

I was asked to speak at one of the early PHA programs for physicians and patients and was so impressed with PHA's extraordinary efforts and success at PH education. In scleroderma, we really need the education to teach the physicians how to identify, screen, diagnose and get the patients treated. Through PHA Online University and other educational programs, we are making more educational opportunities possible. In the future, I look forward to educating even more rheumatologists.

What's the most important piece of advice you give your PH patients?

I encourage all of my PH patients to participate in a cardiopulmonary exercise program to improve their function and to stay as active as they can. As for other scleroderma patients with PH, I would encourage them to work very closely with both the PH doctor as well as their rheumatologist. The scleroderma patient has additional problems besides PH that should not get neglected, and there are scleroderma problems that mimic side effects of medication, so it is important to have a team of doctors who work together to optimize care.

Interview conducted by Rebecca Kurikeshu, PHA Medical Outreach Program Associate


FacebookTwitterInstagram iconLinkedInYouTube

For optimal viewing of, please use a standards-compliant browser such as Google Chrome or Firefox.

The information provided on the PHA website is provided for general information only. It is not intended as legal, medical or other professional advice, and should not be relied upon as a substitute for consultations with qualified professionals who are familiar with your individual needs.

801 Roeder Road, Ste. 1000, Silver Spring, MD 20910   Patient-to-Patient Support Line: 1-800-748-7274
    Privacy Policy    Provide Feedback & Report Bugs

Designed by Matrix Group International, Inc.® | © 2016 Pulmonary Hypertension Association. All Rights Reserved.


The National Organization for Rare Disorders (NORD) awarded PHA the Abbey S. Meyers Leadership Award in 2012 for outstanding service to PHA members in advocacy, education and other key areas.