If You Have Scleroderma, Are You Paying Enough Attention to Your Lungs?

This article is for people who have scleroderma, or their care partner, as well as others who want to learn more about scleroderma. The goal of this patient education activity is to help patients with scleroderma know the symptoms of lung disease to report to their doctors.

You will learn about:

• The connection between scleroderma and interstitial lung disease (ILD)

• Recognizing symptoms of ILD

• The importance of tracking your symptoms

• Tips and tools for tracking your symptoms

• The importance of reporting your symptoms to your doctor

• Questions to ask your doctor

Scleroderma is a chronic (life-long) disease that can be difficult to manage.

Some people with scleroderma only have problems with their skin, but some have problems with their blood vessels or internal organs. This is called "systemic" scleroderma.

When the lungs are affected it is called interstitial lung disease (ILD). ILD is a frequent problem in people who have systemic scleroderma.

ILD happens when a protein called collagen builds up in your lungs and causes scarring, which affects your ability to breathe and can keep you from getting enough oxygen into your bloodstream.

Some medicines may slow down the scarring process and improve symptoms, but the scarring usually can't be reversed.

ILD can make you feel short of breath, especially when you are active.

Having trouble breathing is the main symptom of ILD.

Other symptoms of ILD include:

• Dry cough

• Fatigue (tiredness) or weakness

• Heartburn or pain in the chest

• Loss of appetite or weight

Symptoms may come on slowly, but they get worse over time, and can be life-threatening.

ILD can lead to dangerous conditions, such as

• Heart failure

• High blood pressure in your lungs (pulmonary hypertension)

• Lung failure

This is why it is important to notice the symptoms of ILD when they first happen and report them to your doctor as soon as possible.

Because ILD can look or feel like other lung conditions, it's important to keep a good record of your symptoms. Sharing them with your doctor will help make a diagnosis.

Ways to track your symptoms include using:

• Calendars, diaries, or journals

• Check-lists you can download from the Internet and print out

• Computer or mobile device apps

Use the way you are most comfortable with so you can accurately record all of your symptoms. This will give your doctor a complete view of your condition.

No matter how you record your symptoms -- on paper or electronically -- there are certain pieces of information you'll want to gather:

• What the symptom is

• When it happened (date and time)

• If it is a new symptom

• How long it lasted (minutes, hours)

• How severe it was (very mild, mild, severe, very severe)

• How it affected your daily life (no effect, not much, hard to ignore, serious effect)

By the time you have symptoms of ILD, your lungs have been damaged. This is why you should see your doctor as soon as possible.

Your doctor may run certain tests to find out if you have ILD, including:

• Chest x-ray

• CT scan

• Breathing tests, also called pulmonary function tests (PFTs)

• Lung biopsy, an examination of tissue removed from your lung

After your doctor makes a diagnosis, you may start medicines to help manage your symptoms.

If you have scleroderma you may want to ask your doctor questions about ILD, such as:

• What are my chances of getting ILD?

• How will you know if I have it?

• What symptoms should I tell you about?

• If I have ILD, what treatments are available?

• Is there anything I should be doing -- or not doing -- to help avoid getting ILD?

Congratulations!

You have successfully completed the program: If You Have Scleroderma, Are You Paying Enough Attention to Your Lungs?