This article contains an abstract and full transcript of a symposium which took place on 6th September 2026, as part of the European Respiratory Society (ERS) Congress held in Barcelona, Spain, sponsored by INSMED. The Chairperson was Professor Jean-Luc Vachiery and the speakers were Professors Irene Lang (Medical University, Vienna, Austria) Hossein Ardeschir Ghofrani (Giessen Medical Center, Germany), and Ioana Preston (Lahey Clinic, Massachusetts).
The four experts discussed the role of inhaled treatments for pulmonary arterial hypertension (PAH) and for pulmonary hypertension linked to interstitial lung disease (PH-ILD).
Burden of disease (Vachiery)
- Pulmonary hypertension affects about 1% of the world’s population. Survival in pulmonary arterial hypertension now exceeds five years on average, but the disease remains incurable. Pulmonary hypertension linked to interstitial lung disease is far more serious, with average survival of about 15 months.
- Symptoms are not specific, so diagnosis takes on average 1.4 to 1.6 years, and around 40% of patients are first misdiagnosed.
- Care varies widely across Europe: only 37% of treated patients receive combination therapy, although it is recommended. No treatment is approved in Europe for pulmonary hypertension linked to interstitial lung disease.
- Most patients are already severely limited at diagnosis, and only about a third manage to work full time.
Current treatments (Lang)
- Treatments acting on the prostacyclin pathway are effective but often poorly tolerated: 14 to 19% of patients in trials stopped because of side effects.
- In lung disease, most trials of treatments for pulmonary arterial hypertension have failed, some even causing harm. Inhaled treprostinil is the only treatment approved for this group, in the United States only, so in Europe patients mostly receive sildenafil or tadalafil off-label.
- New treatments are coming: oral ralinepag, taken once a day, reduced the risk of clinical worsening by 55% in a large trial, and a treprostinil skin patch is in early development.
Why inhaled treatments (Ghofrani)
- Inhaled drugs go straight to the lungs and act mainly on the well-ventilated areas, so they cause fewer side effects and don’t worsen oxygen levels, unlike drugs given by tablet or drip.
- Older inhaled treatments (iloprost) required six to nine inhalations a day. Devices have since improved, with quieter, faster nebulisers and small, portable dry powder inhalers.
- New formulations (liposomes, prodrugs, nanoparticles) aim to release the drug slowly so it can be taken less often and irritate the airways less.
Current and future inhaled treatments (Preston)
- Iloprost is approved in Europe for pulmonary arterial hypertension. Inhaled treprostinil, by nebuliser or dry powder inhaler, is approved in the United States for both conditions. Cough is the most common side effect.
- In a large trial in patients with lung disease, inhaled treprostinil improved the walking test by about 31 metres and reduced lung disease flare-ups. Patients strongly preferred the dry powder inhaler to the nebuliser.
- Two new treatments are in development: L606, taken twice a day by nebuliser, and treprostinil palmitil, taken once a day with a dry powder inhaler. In an early trial, treprostinil palmitil reduced resistance in the lung vessels by 35% and improved the walking test by 35 metres, and was generally well tolerated. Large final-stage trials are under way for both conditions.
Overall message: inhaled treatments are more targeted and better tolerated, and the next generation aims to make them easier to take, which matters especially for the lung disease group, where Europe still has no approved treatment.
Read more at this link on the European Medical Journal