Systemic Sclerosis Patients in Australia Not Always Screened for PAH Despite Guidelines

Physician adherence to guidelines for screening for pulmonary arterial hypertension (PAH) in patients with systemic sclerosis (SSc) was found to be poor in Australia, according to a multi-center research study that evaluated the percentage of doctors who adhere to recommended SSc guidelines.

The study, “Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme,” was published in the journal Arthritis Research & Therapy.

PAH is the leading cause of death in SSc patients, and is caused by high blood pressure in the arteries that supply blood to the lungs. Annual screening of SSc patients using an echocardiogram — an ultrasound scan of the heart — is recommended to detect PAH as soon as possible in the course of the disease.

“Diagnosis of early PAH is particularly important as patients with early PAH can progress rapidly, supporting the need for early treatment in this patient population,” the authors wrote.

Of the 1,636 patients with SSc analyzed in the study (recruited from July 2007 and July 2016), 194 (11.9%) had PAH proven by right heart catheterization (RHC – a tiny tube inserted into an artery in the neck that reaches a blood vessel in the heart to measure blood pressure there). Of those 194 patients with PAH, 160 cases were detected by screening at the start of the study or in subsequent screenings.

Patients with PAH detected in later screenings tended to have less advanced PAH than patients whose PAH was detected on the initial screening. This is probably because PAH had already progressed in patients diagnosed on the first screening.

Researchers found that fewer than half of the Australian rheumatologists conducted an annual PAH screening using an ECHO-based algorithm in their patients with SSc — the patients who had been diagnosed with SSc at least 10 years before the study.

The authors noted, however, that “since the establishment of ASCS [the Australian Scleroderma Cohort Study] in 2007, Australian rheumatologists’ adherence to screening guidelines has improved.”

Among rheumatologists who did not perform a transthoracic echocardiogram (the most common type of echocardiogram) to detect PAH in their patients, 50% reported this was due to difficulties in assessing the right heart pressure through the technique. “Referral of these patients to a tertiary screening centre may overcome this obstacle,” the authors suggest.

Other explanations for not following the recommended PAH screening guidelines included the cost of screening (reported by 60% of the rheumatologists) and concerns about how to interpret the results (reported by 80%).

“SSc-PAH is a tragic consequence of SSc, which despite advances in therapy, is the leading cause of SSc-related death. Screening with a web-based algorithm can identify patients with earlier PAH and improve outcomes. Identifying modifiable barriers to screening may improve adherence and ultimately patient outcomes,” the team concluded.