Practitioners at a rural medical center within an academic system sought to compare the risk of lung disease among patients with systemic sclerosis (SSc) to a subset of the EUSTAR cohort and gain understanding of screening practices to identify opportunities for early detection.

Jenna Marinock, MD, from Temple University Hospital in Philadelphia, Pennsylvania, presented the abstract.

Dr Marinock noted that interstitial lung disease (ILD) and pulmonary hypertension (PH) are the most frequent causes of morbidity and mortality among patients with SSc, yet few guidelines exist to guide rheumatologists in screening practices that could help to identify patients early the course of their disease.

In their retrospective analysis of patients with SSc, Dr Marinock and colleagues identified patients aged 18 years and older who had been diagnosed with SSc and seen by a rheumatologist between January 1, 2018, and December 31, 2020. They reviewed pulmonary function tests (PFTs) and transthoracic echocardiogram (TTE) orders during this period for monitoring, focusing on diffusion capacity (DLCO) and pulmonary artery systolic pressure (PASP).

“A total of 244 patients with a SSc diagnosis were seen in a 3-year period, with 187 (over 75%) patients having a visit in the last year,” the authors wrote. Of these patients, 181 (74.2%) had PFTs completed with an average DLCO of 63.6%; 20% of patients had a DLCO less than 50%.

“For TTEs, 189 patients (77.5%) had TTEs ordered with 3 incomplete tests. Of interest, only 99 (53%) had a reported PASP value, and mean PASP was 34.6mmHg (Table 1). Compared to EUSTAR, patients were older (60.3 vs. 56.3 years; p< 0.0001) and mean DLCO values were lower (63.6% vs. 68.7%; p=0.0031). Mean PASP comparatively was 34.6 mmHg vs. 29.8 mmHg (p=0.0009).”

The investigators concluded, “We identified an opportunity for early detection of SSc-ILD and SSc-PH in scleroderma patients within our academic health system. This would be achieved by improving ordering practices of PFTs and TTE for scleroderma monitoring. Additionally, we identified our population as, statistically, having potentially more severe disease compared to the EUSTAR cohort. We intend to investigate these findings further and explore clinical and social factors possibly playing a role in disease progression. We are hoping to institute improvements in best practice and develop EHR toolkits to improve monitoring of our SSc patients in the future.”

—Rebecca Mashaw

Reference:

Marinock J, Nicholas PD, Koons K, Kriplani R, Berger A, Bulbin D. Description of a scleroderma cohort and management of lung disease risk at a rural academic medical center. Presented at: American College of Rheumatology Convergence 2021; November 5-9, 2021; virtual. Abstract 0142.