U-M Scleroderma director takes lead role in new guidelines for screening for PAH in Systemic Sclerosis

All patients with systemic sclerosis (SSc) and connective tissue diseases should be screened for pulmonary arterial hypertension (PAH), according to new recommendations from an international panel of experts that includes a top U-M researcher.

The recommendations were 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.

“These recommendations are the first to provide such specific guidelines that are evidence-driven and consensus-based,” says co-principal investigator, Dinesh Khanna, M.D., M.S., the Marvin and Betty Danto Research Professor of Internal Medicine and director of the U-M Scleroderma Program. “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.”

The article “Recommendations for Screening and Detection of Connective-Tissue Disease Associated Pulmonary Arterial Hypertension,” 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 panel recommends 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: http://www.ncbi.nlm.nih.gov/pubmed/24022584

Read the full story at the Scleroderma Foundation website: http://www.scleroderma.org/site/News2?page=NewsArticle&id=9233

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