Abstract Background/Aims Pulmonary artery hypertension (PAH) is a known complication of systemic sclerosis (SSc). The prevalence is estimated to be between 5-19%, with an annual incidence of developing PAH at approximately 1%. It requires screening as patients are often asymptomatic or symptoms such as dyspnoea can be attributed to more common pathologies. Early detection and treatment have been shown to improve survival outcomes compared to those diagnosed by routine care. The European Society of Cardiology (ESC) guidelines recommend annual screening to be done with echocardiogram, pulmonary function testing (PFT), B-Type natriuretic peptide (BNP) and serum urate. The aim of this review was to assess adherence of our rheumatology centre to the ESC guidelines between the years 2019-2024 and the effectiveness of the screening programme in referring patients on for formal PAH diagnosis. Methods This was a retrospective review of patients’ charts, outpatient letters and investigations. Patients with an established diagnosis of systemic sclerosis prior to 2019 were chosen for review. Any patients with a pre-existing diagnosis of PAH were excluded from analysis. Patients included in the review were required to attend our rheumatology centre from 2019-2024. Data were also collected regarding the number of patients referred for right heart catheterisation and how many of these patients were subsequently diagnosed with PAH. Data collection included antibody status (anti-centromere or anti-SCL70), number of echocardiograms, number of PFTs, serological testing of serum urate and pro-BNP. Results There was a total of 36 patients included in the review. All patients had at least one annual review for PAH. The median number of PFTs and echocardiograms in the five-year period per patient was three. 39% (n = 14) had a pro-BNP and 19% (n = 7) had a urate checked at least once in the 5 years. 25% (n = 9) had a positive anti-SCL 70 and 50% (n = 18) had a positive anti-centromere antibody, with the remaining patients being antibody negative. 19.4% ( n = 7) of patients were referred to cardiology for consideration of right heart catheter. Of those patients who were referred, 14% (n = 5) were diagnosed with confirmed PAH. All five patients diagnosed with PAH were anti-centromere antibody positive. PAH prevalence of 14% in our patient group over 5 years is consistent with international prevalence of 5-19%, suggesting that our screening programme is effective. Conclusion Best practice care of SSc should incorporate a screening program that facilitates a multidimensional PAH risk evaluation at the baseline. This can allow for the initiation of early intervention and treatment, improving survival outcomes. A multimodal approach was used in our centre, which was effective, but access to investigations such as echocardiograms and PFTs was restricted during the COVID-19 pandemic years. Utilisation of the DETECT algorithm or ASIG PAH calculator may be of benefit where access to investigations is restricted. Disclosure D. O Brien: None. S. Harney: None.
Brien et al. (Wed,) studied this question.