Pulmonary arterial hypertension screening practices in scleroderma patients among Canadian rheumatologists

Abstract

Background: Current international guidelines recommend annual screening for pulmonary arterial hypertension with transthoracic echocardiogram and pulmonary function testing in all patients with scleroderma (systemic sclerosis). Our objectives were to determine Canadian rheumatologists’ screening practices for pulmonary arterial hypertension in patients with systemic sclerosis and identify reasons why current guideline recommendations may not be followed. Methods: A survey was emailed to all Canadian Rheumatology Association members. They self-identified as systemic sclerosis experts or non-experts and provided basic demographic data. Participants were asked how frequently they screened with transthoracic echocardiogram and pulmonary function testing and, if applicable, why they did not adhere to recommendations. Results: A total of 71 rheumatologists participated, of whom 43 identified as non-experts. Overall, 81.4% ordered annual transthoracic echocardiogram and 77.6% annual pulmonary function testing. Rates of annual transthoracic echocardiogram testing were similar between experts and non-experts, whereas experts ordered annual pulmonary function testing more often. Clinicians with a higher proportion of systemic sclerosis patients in their practice were more likely to follow guidelines. There was an inverse relationship between years in practice and adherence to screening guidelines. The most common reason for not following screening guidelines was disagreement with recommendations, followed by unfamiliarity with guidelines. Conclusions: Pulmonary arterial hypertension screening rates remain sub-optimal in Canada but have improved since 2012. Failure to adopt guidelines is due to rheumatologists disagreeing with or not knowing current recommendations. Future studies should examine why rheumatologists disagree with guidelines and how to improve awareness.

Attitudes and barriers to pulmonary arterial hypertension screening in systemic sclerosis patients: A survey of UK-based rheumatologists

“…As for the rheumatologists' knowledge concerning screening, half of the respondents identified unfamiliarity with the proposed algorithms as a barrier, which is higher than previously reported in rheumatologists surveyed in Ohio (USA) (33.0%) 34 or Canada (21.6%). 31 Interestingly, we found that one-third of respondents considered that screening requires invasive testing, which may reflect respondents incorrectly conflating or confusing diagnostic testing with screening.…”

“…For example, the rate reported by Morrisroe et al 17 found 84.4% adherence to annual screening guidelines via the algorithm proposed by ASIG; however, the adherence rate to annual screening in non-ASIG rheumatologists was much lower (38.5%-58.9%). Quinn et al 31 found a somewhat lower screening rate (71.9%) for Canadian rheumatologists performing annual TTE and PFT. In contrast, Wigger et al 34 reported a much lower screening rate (58.0%) with both the same annual modalities (TTE and PFTs) in a specialised PH and SSc centre in Ohio, USA.…”

“…The main barriers to screening included organisational and external factors such as difficulty in visualising results from other centres, current (long) waiting lists and limited time during daily clinical practice. Conversely, only 13.5% of Canadian rheumatologists surveyed by Quinn et al 31 reported problems with access to screening investigations as a barrier. However, cost was reported less frequently as a barrier in our study (34.0%) than in the Australian study, 17 which was one of the main barriers identified in their research (60.0%).…”

“…5 Combined models have been proposed to increase the predictive effectiveness of the diagnosis and early identification of SSc-PAH, [25][26][27] such as the DETECT 16,28 and the Australian Scleroderma Interest Group (ASIG) algorithms. 29 Given the significant international variation observed in the approaches adopted to screening, and barriers to facilitating timely screening in routine clinical practice, 17,30,31 the absence of dedicated UK-based guidance is of practical concern. In addition, the current British Society of Rheumatology (BSR) guidelines for of SSc (currently under revision) concerning treatment 32,33 make no specific recommendations concerning either the frequency or specific investigations for SSc-PAH screening.…”

The Relationship between Pulmonary Damage and Peripheral Vascular Manifestations in Systemic Sclerosis Patients

“…The main cause of death in SSc patients is not only collagen overproduction but also the effects collagen overproduction has on the pulmonary system. This includes fibrosis or pulmonary artery hypertension (PAH) [10][11][12][13][14][15]. Recent guidelines have recommend screening with high resolution computed tomography (HRCT) to diagnose interstitial lung diseases (ILD) in SSc patients at the baseline visit and once the diagnosis of ILD has been established [16,17], whilst a combination of HRCT and pulmonary function tests is recommended to quantify the extent and severity of ILD [16][17][18][19].…”