Your PAH may be progressing silently, even when
your symptoms seem stable.1
PAH can involve changes in the levels of three
substances that occur naturally in your body. Over time, these changes can cause the arteries in your lungs
to become thick and stiff. As this happens, the arteries of your lungs may tighten or narrow and scar,
making it more difficult for blood to flow. When blood flow is restricted, your heart must work harder to
pump blood through the arteries of your lungs. This can cause the muscles in your heart to thicken, which
can make it more difficult for your heart to pump blood. As time goes on, the strain on your heart can
Arteries in the lungs may be affected when
there is either too much or too little of three naturally occurring substances in your body, but there are
medications available that can target each of these three key pathways.2
Nitric Oxide Pathway
Phosphodiesterase type-5 inhibitors (PDE-5 inhibitors) work
Endothelin receptor antagonists (ERAs) work here.2
Prostacyclin, prostacyclin analogs, and prostacyclin receptor
agonists work here.2
When you have PAH, your body may produce too little of a substance
called prostacyclin. Prostacyclin activates a process that helps open arteries.2,3
UPTRAVI® (selexipag) works in a similar way to help open the arteries.4
There are steps you can
take that may delay PAH progression and it starts with talking to your doctor.
In a clinical
UPTRAVI® reduced the risk of PAH disease progression* by 40%.4
27% of patients treated with UPTRAVI® experienced a PAH disease progression event vs 41.6%
of patients not taking UPTRAVI®.4
UPTRAVI® reduced the risk of PAH-related hospitalizations. This benefit was demonstrated in
a clinical trial comparing patients taking UPTRAVI® to those not taking UPTRAVI®. Patients took UPTRAVI®
for an average of 1.4 years.4
In this same clinical trial, patients taking UPTRAVI® walked an average of 12 meters (about
39 feet) farther than patients not taking UPTRAVI®, as measured by the 6-minute walk distance
*In a clinical trial of UPTRAVI®, PAH disease progression was defined as a serious event,
such as hospitalization for PAH; the need to start injectable PAH medications or chronic oxygen therapy;
lung transplantation; a decline in 6-minute walk distance (6MWD) combined with worsening of functional
class (FC) or need for additional PAH therapy; or death.
How the study was conducted:
UPTRAVI® was studied in the largest trial of any
FDA-approved PAH treatment4
The trial included 1156 total PAH patients.
Compared 574 patients taking UPTRAVI® with 582 patients taking placebo (a sugar pill)
80% of enrolled patients were treated with an endothelin receptor antagonist (ERA),
phosphodiesterase type-5 inhibitor (PDE-5 inhibitor), or both for PAH
20% of enrolled patients were not on any PAH treatment
Nearly all patients were FC II or FC III (PAH patients range from FC I to FC IV)
Patients taking UPTRAVI® were treated for an average of 1.4 years during this study
The trial included the most scleroderma-related PAH patients of any PAH trial (170 out of
You may be able to delay PAH progression. Don't delay in asking your doctor how.
An aggressive disease like PAH may call for an
aggressive treatment approach. Learn how to discuss UPTRAVI® with your doctor and start exploring an
additional angle to help delay PAH disease progression by downloading this doctor discussion guide.
In a clinical study of UPTRAVI®, PAH disease progression was
defined as a serious event, such as hospitalization for PAH; the need to start injectable PAH medications or
chronic oxygen therapy; lung transplantation; a decline in 6-minute walk distance (6MWD) combined with
worsening of functional class (FC) or need for additional PAH therapy; or death.
References: 1. Lau EM, Humbert M, Celermajer DS.
Early detection of pulmonary arterial hypertension. Nat Rev Cardiol.
2015;12(3):143-155. 2. Humbert M, Lau EM, Montani D, Jaïs X, Sitbon O,
Simonneau G, et al. Advances in therapeutic interventions for patients with pulmonary arterial hypertension.
Circulation. 2014;130(24):2189-2208. 3. McLaughlin
VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. Circulation. 2009;119(16):2250-2294. 4.
UPTRAVI® (selexipag) full Prescribing Information. Actelion Pharmaceuticals US, Inc. 5. Gaine S, Chin K, Coghlan G, et al. Selexipag for the treatment of connective
tissue disease-associated pulmonary arterial hypertension. Eur Respir J.
2017;50(2). 6. Aithala R, Alex A, Debashish D. Pulmonary hypertension in
connective tissue diseases: an update. Int J Rheum Dis. 2017;20:5-24.
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