Biologic Therapies Summit
Biologic Therapies Summit
May 22, 2019
3 min read

Patients with scleroderma require 'annual screening' for pulmonary arterial hypertension

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Virginia Steen

CLEVELAND — Every patient with systemic sclerosis should be screened for pulmonary arterial hypertension every year, as early recognition, referral and aggressive treatment are necessary to improve long-term outcomes, according to Virginia Steen, MD, of the Georgetown University Medical Center.

“Pulmonary hypertension is common in rheumatic disease and, according to the REVEAL Registry, about 25% of all pulmonary arterial hypertension is related to connective tissue diseases,” Steen told attendees at the Biologic Therapies Summit. “When they broke that down, 62% were because of scleroderma.”

“Now, remember this [study] is out of the pulmonary world, where there is not always a rheumatologist, so it was unclear how many of the lupus or [mixed connective tissue disease] patients were also in the scleroderma spectrum or not,” she added. “However, scleroderma does occur in lupus and all the connective tissue diseases, just much less commonly than in scleroderma.”

Every patient with systemic sclerosis should be screened for pulmonary arterial hypertension every year, according to Steen.
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However, the challenge in identifying pulmonary arterial hypertension is that all patients with connective tissue disease, including systemic sclerosis, could have a variety of reasons for dyspnea. In addition to pulmonary hypertension, these include interstitial lung disease, left heart disease, anemia and musculoskeletal problems, as well as deconditioning, aging or depression.

“It is very challenging, and really important that we focus on understanding with our patients how much exercise they can do, how much things have changed over time, and whether there really is a component of dyspnea and decreased endurance in their ability to exercise. These patients have a tendency to adapt to their functionality — many patients just walk slower, don’t carry groceries and don’t go to church because they would have to climb stairs — so we really have to work at getting the history of dyspnea from these patients.”

According to Steen, clinical risk factors for pulmonary arterial hypertension in scleroderma include longer-duration Raynaud’s phenomenon, older-onset systemic sclerosis, being post-menopausal and having limited cutaneous disease, as well as cutaneous telangiectasias and severe, new digital ischemia.

Other reliable predictors of the risk for pulmonary arterial hypertension include the presence of anti-centromere antibodies, as well as the noninvasive pulmonary function test, she said. A diffusing capacity of the lung for carbon monoxide (DLCO) of less than 60% should be considered predictive, as only about 5% to 10% of patients with pulmonary arterial hypertension related to systemic sclerosis have a DLCO of more than 70%.

In screening patients for pulmonary hypertension, the echocardiogram is the test of choice, according to Steen. Echocardiogram signs that indicate symptomatic pulmonary arterial hypertension include increased systolic pulmonary arterial pressure or tricuspid regurgitation velocity, and the flattening of the intra-ventricular septum. Other signs include a small left ventricle dimension and dilation of the pulmonary artery.


However, Steen cautioned that echocardiogram reports do not always mention the systolic pulmonary arterial pressure. Further, pulmonary arterial pressure readings of 30 mmHG to 50 mmHg in echocardiograms are often unreliable, she added.

“Be careful and actually look at the echocardiogram report,” Steen said. “Even if we rheumatologists aren’t great at reading echocardiogram reports, you can work with the cardiologist to review it.”

According to Steen, the three screening models for pulmonary arterial hypertension in patients with systemic sclerosis are the 2015 European Society of Cardiology/European Respiratory Society Guidelines, the DETECT Algorithm — which is not available in the United States — and the Australian Scleroderma Interest Group Screening Protocol.

“Pulmonary arterial hypertension is a major complication of scleroderma, and we as rheumatologists must identify these patients early,” Steen said. “Whatever you want to use as a screening tool is OK, but make sure you do it, identify these patients early and get them treated. Work with your colleagues and be aggressive, and give your patients a better life expectancy. I really think we have turned this from a deadly disease into a treatable disease.” – by Jason Laday


Steen V. Pulmonary hypertension — when to screen, how to treat in rheumatic diseases. Presented at: Biologic Therapies Summit VIII; May 16-17, 2019; Cleveland, Ohio.

Disclosure: Steen reports consulting fees from Bayer and REATA Pharmaceuticals.