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Consensus Guidelines Published on Screening for PAH in Systemic Sclerosis

Recommendations Aim for Earlier Diagnosis of This Leading Cause if Mortality


Christina Relacion, Communications Manager, Scleroderma Foundation
(800) 722-4673, Ext. 243

Mollie Katz, VP for Community Engagement, Pulmonary Hypertension Association
(301) 565-3004 x774 or (240) 330-5638

(Oct. 3, 2013) – All patients with systemic sclerosis (SSc) and connective tissue diseases should be screened for pulmonary arterial hypertension (PAH), an international panel of experts recommended in an article published in the September issue of the journal Arthritis & Rheumatism.  The recommendations, part of a larger effort to improve patient outcomes by diagnosing earlier, identify specific tests to be performed in screening these patients for PAH.

PAH is a rare, chronic and progressive lung disease, often difficult to diagnose.  PAH affects up to 15 percent of those with CTD and is the leading cause of mortality among them. SSc, or scleroderma, is a chronic connective tissue disease generally classified as one of the autoimmune rheumatic diseases. Both are complicated conditions.

The article was the result of a literature review and consensus process by CTD and PAH experts from the United States, Canada and the European Union. The panel was convened by the Pulmonary Hypertension Association (PHA) and the Scleroderma Foundation, Inc.

The article, “Recommendations for Screening and Detection of Connective-Tissue Disease Associated Pulmonary Arterial Hypertension,” recommended the following initial screening evaluation of CTD patients:

  • Pulmonary function test (PFT) including diffusion capacity carbon monoxide (DLCO)
  • Transthoracic echocardiogram (TTE)
  • N-terminal pro b-type natriuretic peptide (NT-Pro BNP)

The recommendations also called for annual TTE and PFT in SSc and spectrum disorders and for the full screening panel to be performed as soon as any new signs or symptoms are present.

An abstract of the journal article can be read here:

These recommendations are the first to provide such specific guidelines that are evidence-driven and consensus-based, said the article’s co-principal investigator, Dinesh Khanna, M.D., of the University of Michigan in Ann Arbor.  “Earlier recommendations that were developed as part of larger efforts in PAH did not provide detailed recommendations for patients with CTD-PAH.  Hence, our efforts to develop these recommendations for screening and early detection of CTD-associated PAH,” said Khanna.

PHA’s President and CEO, Rino Aldrighetti, said, “Our goal was to bring renowned clinicians and researchers together to collaborate on these guidelines, taking into consideration different screening and diagnosis practices internationally.”  

Robert Riggs, CEO of the Scleroderma Foundation, said “Scleroderma is among the most complex of connective tissue diseases. The Scleroderma Foundation, PHA and the medical experts involved in this project agree that these guidelines will arm the medical community with a unified approach to caring for patients living with these complicated conditions.”

PHA has made earlier diagnosis a high priority now that multiple treatments are available for PH and additional treatments are undergoing review by the Food and Drug Administration. The Scleroderma Foundation is one of 33 organizations around the world who endorse PHA’s Sometimes its PH early diagnosis campaign (

Funding for the consensus process was provided to the Scleroderma Foundation and PHA by Actelion Pharmaceuticals, US, Inc.; Gilead Sciences, and United Therapeutics Corporation, through unrestricted medical education grants. Dr. Khanna was also supported by a grant from NIH/NIAMS.

About The Pulmonary Hypertension Association (PHA)

Headquartered in Silver Spring, Md., PHA is the country’s leading organization connecting pulmonary hypertension patients, families, and medical professionals. Its mission is to find ways to prevent and cure pulmonary hypertension and provide hope for the community through support, education, research, advocacy and awareness. PHA provides free access to information on its Web site about pulmonary hypertension and facilitates over 200 support groups around the nation, helping many of the estimated 30,000 diagnosed patients in the U.S. To learn more, visit:

About The Scleroderma Foundation

The Massachusetts-based Scleroderma Foundation is the world’s largest nonprofit organization focused on the autoimmune disease scleroderma (a.k.a. systemic sclerosis).  It has a three-fold mission to provide patient education services, generate greater awareness of the disease, and to fund medical research to find the cause, better treatments, and a cure.  The Foundation’s national network of 24 chapters and more than 160 active support groups connect patients, families and the medical community, and serves to advance the Foundation’s mission to the 300,000 people living in the United States with the disease.  For more information about scleroderma and the Scleroderma Foundation, visit:


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