Scleroderma and the Risk of PAH

KNOWLEDGE I N A C T I O N

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Learning about scleroderma

At Actelion, we believe that knowledge is power. When you understand your condition, you have the power to ask the right questions, make informed decisions, and get the most from your healthcare team and your therapeutic options. That’s our goal in PAH education and that’s KNOWLEDGE IN ACTION.

Pulmonary arterial hypertension (PAH) is a well-known complication in people living with connective tissue diseases• Scleroderma is a type of connective tissue disease2

• Scleroderma is also sometimes called systemic sclerosis3

Scleroderma is a type of connective tissue diseaseConnective tissue diseases are a group of diseases that affect blood vessels and connective tissues, such as3:

• Muscles • Cartilage • Tendons • Skin • Ligaments

These diseases may appear in one or more areas of the body. Some examples of connective tissue diseases are3:

• Rheumatoid arthritis • Systemic sclerosis • Vasculitis• Systemic lupus erythematosus (lupus)

Systemic sclerosisScleroderma, also known as systemic sclerosis, affects the blood vessels and connective tissues of the body. This disease causes hardening of the skin, scar tissue buildup, and organ damage.4,5

Between 8% and 12% of all scleroderma patients develop PAH1

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K N O W L E D G E I N A C T I O N : S C L E R O D E R M A A N D T H E R I S K O F P A H

What you need to know about PAH

PAH and the right side of the heart

• With the higher blood pressure caused by PAH, the right side of the heart must work harder to pump blood through the pulmonary arteries8

• The strain of this overload on the heart can lead to right heart failure8

Pulmonary arteries are a type of blood vessel

Pulmonary arterial hypertension (PAH) affects the arteries that pump blood from the heart to the lungs (pulmonary arteries), causing them to become stiff and thick. As the disease worsens, the thickened artery walls form scar tissue.6

These changes make it difficult for blood to flow. The result is PAH— a serious condition that causes continuous high blood pressure in the lungs.7

Healthy artery

Initial signs of PAH

Worsening signs of PAH

Common symptoms of PAH9

• Unexplained shortness of breath

• Constant tiredness

• Chest pain or discomfort

• Light-headedness and fainting (also known as syncope)

• Swelling of the ankles, legs, abdomen, or arms (also known as edema)

These symptoms may be mild at first, but they might get worse over time. If you experience these symptoms and think you may have PAH, you should contact your doctor.

Diagnosis is important. The sooner you are diagnosed, the sooner your doctor may be able to help manage your PAH.

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K N O W L E D G E I N A C T I O N : S C L E R O D E R M A A N D T H E R I S K O F P A H

Right heart catheterization is the most accurate test for PAH and provides more information about the heart’s condition1 This test involves passing a thin tube (catheter) into the right side of the heart in order to test heart function and measure blood pressure in the arteries of the lung. Right heart catheterization is required for a definitive diagnosis of PAH.12

Once symptoms are detected, these tests can help determine if you have PAH

Screening is a basic tool doctors use to check your health when you are at risk for a disease. There are a few simple tests your healthcare team may recommend.1

Screening for PAHYour healthcare teamEvery situation is unique. Your doctor will work closely with you and your caregivers to create a plan that is best for you. Your healthcare team will work together to find out if you have PAH. Your team may include:

• Rheumatologist• Heart doctor (cardiologist) or lung doctor (pulmonologist) to diagnose and

treat your PAH• Nurses to help manage your care and to answer any questions you may have• Nutritionist, in case you need help managing a diet recommended for

PAH patients – Fluid retention is problematic for patients with PAH, so you may need

to reduce the amount of sodium in your diet13

• Social worker, to help address life challenges and support well-being

Diagnostic test Purpose of test

Echocardiogram This test uses sound waves to create images of the heart.10 It enables your healthcare team to see how well your heart is beating and pumping blood.

Electrocardiogram (ECG/EKG)

This test records the heart’s electrical activity. It also shows whether your heart’s rhythm is steady or irregular and may show if your right ventricle is enlarged or strained.11

Pulmonary function test (PFT)

PFTs are breathing tests that measure how much air the lungs can hold, how well they move air, and how well they supply the body with oxygen.12

REFERENCES1. Pulmonary Hypertension Association. Scleroderma and Pulmonary Hypertension. https://www.phassociation.org/document.doc?id=914. Accessed April 25, 2017. 2. Galiè N, Manes A, Farahani KV, et al. Pulmonary arterial hypertension associated to connective tissue diseases. Lupus. 2005;14:713-717. 3. Taber’s Medical Dictionary Online. Connective tissue disease. http://www.tabers.com/tabersonline/view/TabersDictionary/763294/0/connective_tissue_disease. Accessed April 25, 2017. 4. Pulmonary Hypertension Association. PH and Scleroderma. https://www.phassociation.org/AssociatedDiseases/Scleroderma. Accessed April 25, 2017. 5. Genetics Home Reference. Systemic scleroderma. https://ghr.nlm.nih.gov/condition/systemic-scleroderma. Accessed April 25, 2017. 6. Gaine SP, Rubin LJ. Primary pulmonary hypertension. Lancet. 1998;352:719-725. 7. McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension. J Am Coll Cardiol. 2009;53:1573-1619. 8. McLaughlin V. Pulmonary hypertension. In: Goldman L, Schafer AI, eds. Goldman’s Cecil Medicine. 24th ed. Philadelphia, PA: Elsevier; 2012:389-397. 9. Gibbs JSR. Making a diagnosis in PAH. Eur Respir Rev. 2007;16:8-12. 10. Dorland’s Illustrated Medical Dictionary. 32nd ed. Philadelphia, PA: Elsevier Saunders; 2012. 11. How is pulmonary hypertension diagnosed? National Heart, Lung, and Blood Institute website. https://www.nhlbi.nih.gov/health/health-topics/topics/pah/diagnosis. Updated August 2, 2011. Accessed April 25, 2017. 12. Hayes GB. How do I really know I’ve got PH? In: Hayes GB. Pulmonary Hypertension: A Patient’s Survival Guide. 5th ed. Silver Spring, MD: Pulmonary Hypertension Association; 2013:chap 2. 13. Pulmonary Hypertension RN. Salt and Water Restrictions. http://pulmonaryhypertensionrn.com/salt-and-water-restrictions-for-ph -patients/. Accessed April 25, 2017. 14. Hoeper MM, Bogaard HJ, Condliffe R, et al. Definition and diagnosis of pulmonary hypertension. J Am Coll Cardiol. 2013;62(suppl):D42-D50.

Actelion provides additional resources and support through:PAHumanwww.pahuman.com

The following organizations offer more information:Pulmonary Hypertension Association Scleroderma Foundationwww.phassociation.org www.scleroderma.org1-301-565-3004 1-800-722-4673

Actelion and the PAH community are here to help

© 2017 Actelion Pharmaceuticals US, Inc. All rights reserved. ACT-01549 0617

It’s important to take charge of your overall health• Patients with scleroderma should be screened every year for PAH—

even if they do not have symptoms14

• If you’re not sure if you’ve been screened for PAH, talk to your doctor