Who is at higher risk for PAH?

People who have certain diseases, such as scleroderma, congenital heart disease, sickle cell disease, or HIV infection, are at higher risk of developing pulmonary arterial hypertension (PAH). In addition, using certain drugs or being exposed to certain toxins can also increase the chances of developing PAH. Finally, when someone in your family has or had PAH, there is a greater chance that you will also. Keep in mind that these are only risk factors and do not mean that you will necessarily develop PAH.

Scleroderma and PAH

Scleroderma is a disease that can cause thickening, hardening, or tightening of the skin, blood vessels, and internal organs. There are 2 main types of scleroderma, one that affects only the skin and another—systemic sclerosis—that can affect any part of the body. Between 8-27% of patients with systemic sclerosis may also develop PAH.¹

People who have or suspect they have systemic scleroderma should speak with their healthcare team about getting tested for PAH. Early diagnosis and treatment are important because without treatment, people who have both scleroderma and PAH may decline quickly. Yearly echocardiogram (or echo) screening is recommended for patients with scleroderma to help rule out PAH.¹

Congenital heart disease and PAH

Congenital heart disease is a problem with the heart's structure and function that a person is born with. Between 12-34% of patients with a congenital heart defect may develop PAH. ² One of the most common congenital heart defects is a hole in the wall between the right and left ventricles of the heart. When this hole is large (>1.5cm), up to 50% of patients may develop a severe form of PAH called Eisenmenger syndrome.³

Sickle cell disease and PAH

Sickle cell disease is an inherited blood condition that affects red blood cells. The cells can become rigid and sickle- or crescent-shaped. This decreases their ability to move oxygen through small blood vessels and carry it throughout the body. Some patients with sickle cell disease may develop PAH.

HIV/AIDS and PAH

The human immunodeficiency virus (HIV) weakens the immune system and makes it harder for the body to fight off infections. Acquired immunodeficiency syndrome (AIDS) is the final stage of HIV infection.

People with HIV have an increased risk of developing PAH. It is estimated that 1 out of every 200 people with HIV infection has PAH.¹

Drug abuse/toxins and PAH

Certain toxic agents increase the risk of developing PAH.⁴ Some diet drugs that work by curbing the appetite have been associated with development of PAH.⁵ In addition, the use of amphetamines, methamphetamine, cocaine, and L-tryptophan may also increase the risk of developing PAH.^4,6

IMPORTANT SAFETY INFORMATION
Because of the risks associated with Tracleer, this medication may be prescribed by your doctor only through the Tracleer Access Program.

Liver damage: Tracleer can cause serious liver damage, including in rare cases liver failure and, in situations with regular liver testing, unexplained liver cirrhosis. So you must have a blood test to check your liver function before you start Tracleer and each month after that. Call your doctor right away if you have any of these symptoms of liver problems: nausea, vomiting, fever, unusual tiredness, abdominal (stomach area) pain, or yellowing of the skin or the whites of your eyes (jaundice).

Serious birth defects: Tracleer is very likely to cause serious birth defects. It is important not to be or to become pregnant while taking Tracleer or one month after stopping treatment and to have a pregnancy test before you start the drug and each month thereafter. It is very important for you to use reliable forms of birth control while taking Tracleer. Hormonal contraceptives should not be used alone because they may not be effective in patients taking Tracleer.

Do not take Tracleer with glyburide because it may increase your chance of liver injury. Do not take Tracleer with cyclosporine-A because it may increase your chance of side effects.

Please see Additional Safety Information.
Please see full Prescribing Information, including BOXED WARNING and Medication Guide.

Learn more about how PAH may affect you

REFERENCES:

1. McLaughlin VV. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009;53:1573-619.
2. Engelfriet PM, Duffels MGJ, Möller T, et al. Pulmonary arterial hypertension in adults born with a heart septal defect: The Euro Heart Survey on adult congenital heart disease. Heart. 2007;93:682-687.
3. Simonneau G, Galiè N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004;43(12 suppl S):5S-12S.
4. Simonneau G, Robbins IM, Beghetti M, et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2009;54:S43-54.
5. Rich S, Rubin L, Walker AM, et al. Anorexigens and pulmonary hypertension in the United States: results from the surveillance of North American pulmonary hypertension. Chest. 2000;117:870-874.
6. Chin KM, Channick RN, Rubin, LJ. Is methamphetamine use associated with pulmonary arterial hypertension? Chest. 2006;130:1657-1663.