Home > Could I Have PAH? > Who is at higher risk for PAH?

People who have certain diseases, such as scleroderma, HIV, sickle cell disease, or congenital heart disease, are at higher risk of developing pulmonary arterial hypertension (PAH). In addition, using certain drugs or being exposed to certain toxins can also increase your chance 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 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. About 8-27% of patients with systemic sclerosis also develop PAH.1,2 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.2,3,4 Congenital heart disease Congenital heart disease is a problem with the heart's structure and function that a person is born with. 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, up to 50% of patients may develop PAH, and half of these may develop Eisenmenger syndrome.5 Sickle cell disease 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 through small blood vessels and carry oxygen. About 20% to 40% of patients with sickle cell disease develop PAH.6 HIV infection HIV infection is a disease caused by the human immunodeficiency virus (HIV). HIV weakens the immune system and makes it harder for the body to fight off infections. People with HIV have an increased risk of developing PAH. One out of every 200 people with HIV infection has PAH.7 Drug abuse/toxins Certain diet drugs that work by curbing the appetite have been associated with development of PAH. In addition, the use of amphetamines, cocaine, and L-tryptophan may also increase the risk of developing PAH.4,8

IMPORTANT SAFETY INFORMATION

Because of the risks associated with Tracleer, this medication may be prescribed only by your doctor 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 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 or cyclosporine-A because they may increase your chance of side effects.

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

	What is PAH?
	World Health Organization (WHO) Category 1: PAH

REFERENCES

1. Braunwald E, Zipes DP, Libby P, eds. Heart Disease. 2 vols. 6th ed. Philadelphia, Pa: WB Saunders Co; 2001:1912-1928.

2. Wigley FM, et al. The prevalence of undiagnosed pulmonary arterial hypertension in subjects with connective tissue disease at the secondary healthcare level of community-based rheumatologists (the UNCOVER study). Arthritis Rheum. 2005;52:2125-2132.

3. Koh ET, et al. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol. 1996;35:989-993.

4. Rich S. ed. Primary pulmonary hypertension; executive summary. World Symposium-Primary Pulmonary Hypertension. Evian France; September 6-10, 1998.

5. Simonneau G, Galič N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coli Cardiol. 2004;43(12 suppl S):5S-12S.

6. Lin EE, Rodgers GP, Gladwin MT. Hemolytic anemia-associated pulmonary hypertension in sickle cell disease. Curr Hematol Rep. 2005;4:117-125.

7. Speich R, Jenni R, Opravil M, Jaccard R. Regression of HIV-associated pulmonary arterial hypertension and long-term survival during antiretroviral therapy. Swiss Med Wkly. 2001;131:663-665.

8. 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.