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Pulmonary arterial hypertension treatment goals are changing

At the ISR 2016 Autumn Meeting, Prof Gaine delivered a presentation on the current management of pulmonary hypertension and new developments in the field. 

Prof Gaine said it was important to remember that not all pulmonary hypertension is PAH, stressing PAH is defined by absence of left-heart disease and the absence of parenchymal lung disease. 

He also stated that scleroderma patients are the most common patients seen in clinics who suffer from PAH.

“We used to develop all our drugs in PAH using the six-minute walk distance,” according to Prof Gaine.

“All of the drugs that we currently have more or less have come about as a result of positive outcomes, improvement in walk distance. If you look carefully at it, the scleroderma patients don’t improve very well. The walk distances are poor to begin with; they don’t get a particularly good response on it.”

Prof Gaine said there is more of a focus now on long-term outcome studies, looking at changes to morbidity and mortality, hospitalisation and disease progression when treating the condition.

He added that in treatment plans, patients are now risk-stratified and frequently are put on combination therapy as first-line treatment.

“When we have risk-stratified the patient, if they are very mild, we may go with monotherapy, but increasingly we don’t,” Prof Gaine told delegates.

“A bit like yourselves, we go with initial combination therapy. So we use upfront endothelin receptor antagonists and our PD5 inhibitors. We use them both upfront. Not on the same day, not even on same day, maybe not in the same week, but certainly in the first month. If we start somebody on a monotherapy, we may go to a sequential quickly enough. There are ways of upscaling people who started on monotherapy. But in general, we go now with combination therapy.”

Prof Gaine also said that immunosuppressive therapy is of benefit in some forms of connective tissue disease (CTD)-PAH.

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