It’s challenging to live with a rare disease. When faced with uncertainty about symptoms, support and treatment options, it’s critical to seek out information and resources that can help you take control of your health.
People living with scleroderma, or systemic sclerosis (SSc), understand this well. This condition is a type of connective tissue disease (CTD) that can impact a person’s skin, internal organs, bones and other connective tissues.
While SSc poses challenges of its own, many patients aren’t aware of the connection between SSc and pulmonary arterial hypertension (PAH) — a rare, progressive disease that causes narrowing of the arteries from the right side of the heart to the lungs, leading to elevated pressure in the lungs. SSc can cause inflammation, which may then increase your chances of developing PAH.
Am I at Increased Risk?
While PAH can occur in people with many different CTDs, it’s most common in people with SSc. In fact, up to 19% of people with SSc will develop PAH. Several factors have been associated with increased risk of PAH-SSc, including age and sex — females and people over the age of 50 may be most at risk of developing PAH associated with CTDs such as SSc. Although PAH can affect people of all races, African Americans may be more likely to develop PAH-SSc.
What Symptoms Should I Look For?
Noticeable signs of PAH-SSc — which may start out mild and get worse over time — include light-headedness, shortness of breath, chest pain, fainting, fatigue and swelling of the abdomen, ankles or legs (also called edema).
Because PAH-SSc can begin before you notice it, annual screening is an important tool that can help your doctor detect possible signs of disease. The earlier you’re diagnosed with PAH-SSc, the sooner your doctor can help determine the right treatment plan.
What Else Can I Do?
The best way to take control of your health is by talking to your doctor about your risk of developing PAH-SSc. You can also visit www.UnmaskPAH.com for more information about who is at risk for PAH, the importance of routine screening, and a downloadable discussion guide with questions to consider asking your doctor.
Being in the driver’s seat of your health means listening to your body, being aware of your symptoms and how you’re feeling, and communicating openly and often with your care team about screening and treatment options. Understanding your potential risk of PAH-SSc — and what you can do about it — is part of that journey.
Sponsored by the Janssen Pharmaceutical Companies of Johnson & Johnson.
The Editorial Team at Lake Oconee Health is made up of skilled health and wellness writers and experts, led by Daniel Casciato who has over 25 years of experience in healthcare writing. Since 1998, we have produced compelling and informative content for numerous publications, establishing ourselves as a trusted resource for health and wellness information. We aim to provide our readers with valuable insights and guidance to help them lead healthier and happier lives.