A European Respiratory Society Task Force has updated its pulmonary arterial hypertension treatment guideline using GRADE methodology, addressing four PICO questions. The central recommendation is add-on sotatercept — an activin signalling inhibitor working through a pathway distinct from the three established ones — for patients already receiving PAH drugs who sit at intermediate-low, intermediate-high or high risk of death at follow-up. The certainty of evidence is graded high.
The threshold is what has moved. Intermediate-low risk is not a sick patient by PAH standards; it is a patient who has responded reasonably to initial therapy and is being reviewed. Bringing a fourth agent into that group makes sotatercept part of routine escalation rather than a salvage option.
The Task Force declines to recommend either way in low-risk patients, and says why: low certainty of evidence and few such patients in the randomised trials. That is a more honest answer than extending the recommendation by extrapolation, and it leaves a genuine clinical gap for the best-responding patients.
Two conditions attach. Sotatercept should be given in pulmonary hypertension centres with regular monitoring, and the guideline separately suggests right heart catheterisation during follow-up in the same three risk strata where a therapeutic consequence is expected — which is a reminder that risk stratification in PAH is invasive when it needs to be, not a questionnaire exercise.
- Restratify PAH risk at each follow-up rather than at diagnosis alone; the recommendation is keyed to follow-up risk
- Refer to a pulmonary hypertension centre before starting sotatercept, as the guideline specifies
- Consider repeat right heart catheterisation where the result would change treatment, in intermediate-low risk and above
- Do not extend the sotatercept recommendation to low-risk patients; the Task Force explicitly declined to
- Check current availability and cost in India before discussing sotatercept with a patient
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