From the Journals

Composite screening measures advocated for asymptomatic PAH


Key clinical point: Strategies for early detection of pulmonary arterial hypertension (PAH) are improving with new tools.

Major finding: Screening algorithms are improving sensitivity and specificity for PAH in patients with connective tissue diseases.

Study details: Systematic literature review.

Disclosures: Dr. Khanna has financial relationships with many companies that produce drugs for PAH but no conflicts relative to this study.

Source: Young A et al. Semin Arthritis Rheum. 2018 Oct 14. doi. 10.1016/j.semarthrit.2018.10.010.



In patients at risk for pulmonary arterial hypertension (PAH) due to a connective tissue disease, composite novel screening methods are improving early detection when employed in the context of traditional tools, such as transthoracic echocardiography (TTE), according to a systematic review of studies published over the last 5 years.

The review was conducted to prepare for a guideline update, according to the authors of this recently published summary in Seminars in Arthritis and Rheumatism.

In a literature review for 2012-2015, the authors evaluated whether new tools or strategies have improved PAH screening in patients with connective tissue disease since the last review was undertaken (Semin Arthritis Rheum 2014;43:536-41).

The latest review found that although TTE and pulmonary function tests (PFT) remain a mainstay of screening, there is growing evidence that composite measures, such as the DETECT and ASIG algorithms, add sensitivity and specificity, compared with guidelines that rely on TTE and PFT alone.

After a literature search, the systematic review included 16 cohort studies and 6 case-control studies. Most of these evaluated PAH screening strategies for patients with systemic sclerosis specifically despite the potential for other connective tissue disease etiologies to lead to PAH.

“We need more longitudinal observational studies to develop and validate screening algorithms for non–systemic sclerosis connective tissue diseases,” stated the authors, led by senior investigator Dinesh Khanna, MD, medical director of ambulatory and chronic disease in the University of Michigan’s Office of Research, Ann Arbor.

Relative to screening primarily based on TTE and PFT as advocated in 2009 joint guidelines from the European Society of Cardiology and the European Respiratory Society (ESC/ERS), the preponderance of data supported the addition of DIRECT and ASIG algorithms to improve the sensitivity and specificity of traditional screening and diagnostic tools, according to the data reviewed.

Several of the studies evaluating DETECT and ASIG compared their sensitivities and specificities to the screening strategy recommended in the 2009 ESC/ERS guidelines because the 2015 ESC/ERS guidelines were not yet available at the time these studies were taking place.

In fact, the advantage of these algorithms has been acknowledged in a set of subsequent ESC/ERS guidelines issued in 2015. These specifically recommend DETECT in selected populations, such as adults with more than a 3-year history of systemic sclerosis and less than 60% diffusing capacity of the lung for carbon monoxide.

Based on the systematic review, the authors did not conclude that there is a single set of optimal tests for PAH screening whether in patients with systemic sclerosis or another connective tissue disease, but the authors did conclude that there has been progress in strategies for early PAH detection.

They also speculated that more progress might be coming. They noted that several newer tests, such as stress TTE to assess cardiopulmonary function during exercise, might further improve PAH detection at early stages.

According to the authors, this work is important, because better screening that results in earlier PAH detection means earlier treatment, which, in turn, “may improve survival.”

SOURCE: Young A et al. Semin Arthritis Rheum. 2018; Oct 14. doi: 10.1016/j.semarthrit.2018.10.010.

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