Selexipag in scleroderma-associated pulmonary hypertension: timing and the right ventricle

Design

retrospective multicentre observational cohort, Italy

Population

51 adults with systemic sclerosis-associated PAH confirmed by right heart catheterisation, 94% female, median age 71

Primary outcome

survival, treatment persistence and one-year mortality risk by COMPERA 2.0

Effect

survival 88% at 3 years and 70% at 5 years; low COMPERA 2.0 risk at 12 months in 38% starting within a year of PAH onset versus 8% starting later (p<0.01); persistence 84% at 12 months

Fifty-one Italian patients with systemic sclerosis and pulmonary arterial hypertension confirmed on right heart catheterisation, treated with selexipag for a median of 22 months, were reviewed retrospectively across several centres. Ninety-four per cent were women and the median age was 71.

Survival from PAH diagnosis was estimated at 88% at three years and 70% at five. The only significant protective factor was a lower COMPERA 2.0 risk score. Right ventricular enlargement was associated with higher mortality risk and a higher tricuspid annular plane systolic excursion (TAPSE) with lower risk. COMPERA 2.0 improved in every patient by twelve months, but reaching the low-risk category was much commoner in those who started selexipag within a year of PAH onset — 38% versus 8%. Persistence on the drug was 84% at twelve months, and patients already on combination PAH therapy at baseline were less likely to stop it.

This is 51 patients, retrospective, with no comparison group, so it cannot say that selexipag improved survival. What it does support is that the drug is tolerated over years in this population, and that both the timing of escalation and right ventricular function carry the prognosis. For a rheumatologist that means the screening interval matters more than the choice of agent: SSc-PAH found early enough to escalate within the first year is a different disease from SSc-PAH found late.

• Keep to annual PAH screening in systemic sclerosis — DETECT or ESC/ERS algorithm, not symptoms alone
• Record TAPSE and right ventricular size explicitly when you review the echocardiogram
• Confirm on right heart catheterisation before committing a patient to PAH therapy
• Escalation within a year of PAH onset was associated with reaching low mortality risk
• Selexipag is available in India but costly and not widely reimbursed — raise access early rather than at the point of escalation

The statistics, in plain English

With 51 patients, survival estimates of 88% and 70% carry wide uncertainty and cannot be compared with historical cohorts on other drugs. The adjusted odds ratio of 0.01 (95% CI 0.01-0.27) for right ventricular enlargement is a very unstable estimate — an interval that spans two orders of magnitude reflects few events, so read the direction and not the number. The 38% versus 8% difference in reaching low risk with early treatment is an association: patients treated early may simply have had milder disease found sooner.

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