Evaluating factors influencing screening for pulmonary hypertension in systemic sclerosis: does disparity between available guidelines influence clinical practice?

Pulmonary arterial hypertension (PAH) is one of the leading causes of mortality in systemic sclerosis (SSc). We audited adherence with available recommendations regarding cardiopulmonary screening for PAH in SSc and explored potential factors influencing clinical practice. A retrospective case note review of 108 patients with SSc who had attended outpatient clinic over the previous year was undertaken. Records were scrutinised for evidence of previous assessment with trans-thoracic echocardiography (TTE) and pulmonary function tests (PFT), along with information regarding clinical phenotype and serological subset. The proportion of patients for whom screening had been undertaken within the previous 12 months was low, with significantly fewer having TTE compared with PFT assessment (34.7% vs. 53.1%, p = 0.014). The majority of patients had undergone TTE and PFT assessment within the previous 2 years, but a lower proportion had undergone TTE compared with PFT (69.4% vs. 82.7%, p = 0.044). There were strong trends for more frequent PFT assessment in younger patients, limited cutaneous SSc and worse previous PFT results. In contrast, the frequency of TTE assessment was not associated with previous investigation results or disease subtype. Serological profile did not influence the frequency of either TTE or PFT assessments. Disparity between available published guidelines may influence both the frequency and preference of PAH screening in SSc in clinical practice. The higher frequency of PFT assessment might reflect a perceived superiority amongst clinicians of PFT over TTE in the early identification of SSc-PAH. SSc-specific guidelines, possibly incorporating additional independent risk factors, may improve the cost-effectiveness and clinical efficacy of screening recommendations designed to ensure the early identification of PAH in patients with SSc.