What Is Scleroderma-
Scleroderma-associated interstitial lung disease (ILD) is a potentially serious condition that occurs when scleroderma leads to scarring of the lungs. Before learning more about scleroderma-associated ILD, let's take a closer look at scleroderma.
Scleroderma is an autoimmune disease where the body produces too much inflammation and collagen, which causes thick and rigid scar-like areas, called fibrosis. Fibrosis may take place in the connective tissues that are found in the skin, tendons, cartilage, and internal organs, as well as in the muscles and joints.
Skin symptoms are the most common sign of scleroderma, but this can vary among patients who may also experience additional symptoms in areas like the lungs, heart, kidneys, joints and muscles, and digestive system. Scleroderma may also cause fatigue.
All symptoms, regardless of how insignificant they may seem, should be reported to a healthcare provider.
Diagnosing scleroderma can take several tests and possibly multiple healthcare providers, including specialists who focus on specific areas of the body.
Tests to Diagnose Scleroderma
This is a list of some tests healthcare providers may use to diagnose and monitor scleroderma.
- Physical exam.
- Blood test to look for signs of antibodies associated with scleroderma.
- Urine test to look for the level of a chemical that may indicate how well the kidneys are functioning.
- Skin biopsy.
- Echocardiogram to test the health of the heart.
- Lung health tests, also known as pulmonary function tests, to assess lung function.
- Imaging and scans, such as X-rays, to look at internal organs to assess their health.
Medical Specialists You May See
- Cardiologist – A healthcare provider who specializes in the heart and blood vessels
- Dentist/orthodontist – A healthcare provider who specializes in the teeth and gums
- Dermatologist – A healthcare provider who specializes in the skin
- Dietitian – A specialist who can provide dietary modifications to make eating and digesting easier
- Gastroenterologist – A healthcare provider who specializes in the digestive system
- Obstetrician-gynecologist – A healthcare provider who specializes in women’s reproductive health
- Immunologist – A healthcare provider who specializes in the immune system
- Internist/general practitioner – A healthcare provider who treats non-surgical conditions
- Neurosurgeon – A surgeon who specializes in the brain and nerves
- Podiatrist – A healthcare provider who specializes in the feet
- Physical therapist – A healthcare provider who can help people maintain and gain mobility
- Pulmonologist – A healthcare provider who specializes in the lungs
- Rheumatologist – A healthcare provider who specializes in the joints, muscles, and connective tissues
- Urologist – A healthcare provider who specializes in the urinary system, along with men’s reproductive health
- Nephrologist – A healthcare provider who specializes in the kidneys
What Is ILD and How Is It Related
ILD is a potentially serious condition that affects the lungs and can be caused by known and unknown factors, including some diseases. Scleroderma is one of the diseases known to potentially cause ILD. When scleroderma causes ILD, it may be referred to as scleroderma-associated ILD.
Scleroderma-associated ILD can lead to scarring of the lungs (also known as pulmonary fibrosis) that may worsen over time and—eventually—could make breathing difficult.
What Are the Risks of Developing Scleroderma-Associated ILD?
- Up to 80% of people with scleroderma have some evidence of lung fibrosis. Of these 80%, 25-30% develop progressive ILD.
- People with scleroderma-associated ILD may be asymptomatic and not experience any symptoms in the early stages of ILD, which can delay detection of it.
- ILD is most commonly found in the first few years from diagnosis but can occur at any time.
What Are the Symptoms of Scleroderma-Associated ILD?
Shortness of breath
Feeling breathless during activity or even at rest.
Persistent, dry cough
A cough that doesn’t produce phlegm and won’t go away.
Feeling tired—even after light activity.
Unexplained weight loss
Weight loss that can’t be attributed to diet or exercise.
Clubbing or widening of the fingertips
Fingertips widen—making them appear rounder than usual.
Crackling breath sounds
Lungs may make a Velcro-like tearing sound when heard through a stethoscope.
Muscle and joint pain
Over time, muscles and joints may become painful.
Monitoring for Scleroderma-Associated ILD
Changes in your symptoms can indicate to your healthcare team if your disease is worsening. Talk to your healthcare team about your symptoms and tell them about any changes you notice. Remember that they can best advise you and may be able to help you with some of these.
Tracking Your Symptoms
Watch for any worsening symptoms. These can include:
- Shortness of breath, especially with mild physical activity
- A cough that doesn’t get better
- Tiredness and generally feeling unwell
- Gradual, unintended weight loss
- Rapid, shallow breathing
Remember to bring your completed symptom tracker with you to your next appointment. Symptom monitoring is a powerful tool that may help you have informed discussions with your healthcare provider and healthcare team.
Communicating with Healthcare Providers
Scleroderma-associated ILD requires open, honest communication with your healthcare providers. Report any worsening or new symptoms and concerns—even if you think something is insignificant, your healthcare provider may see it as vital information. Your healthcare provider needs to know as much as possible so you can receive the proper treatment.
Tests for Scleroderma-Associated ILD
We know that getting diagnosed with scleroderma-associated ILD can be a long and sometimes challenging experience, but arming yourself with information about the diagnostic process may help. Continue reading below to learn about the importance of early diagnosis, how scleroderma-associated ILD may progress over time, how to take an active role in the diagnostic process, and information about the tests used to reach a diagnosis.
Why Does Early Diagnosis Matter?
Catching scleroderma-associated ILD early is important because:
- Scleroderma-associated ILD may cause scarring of the lungs (commonly called pulmonary fibrosis), which can progress or get worse over time. You may hear this referred to as having a chronic ILD with worsening fibrosis.
- As scleroderma-associated ILD progresses, the scarring of the lungs becomes worse and breathing may become harder.
- Over time, this may make even the simplest everyday activities, like walking or eating, feel challenging.
- The earlier an accurate diagnosis is made, the earlier you and your healthcare provider can evaluate your management options.
How to Take an Active Role in the Diagnostic Process
Because scleroderma-associated ILD isn’t widely known, it can be easy to confuse the warning signs with other diseases. That’s why it’s important to educate yourself about the disease so you can recognize early indications of scleroderma-associated ILD and talk to your healthcare provider.
Don’t Brush Off Symptoms
The symptoms of scleroderma-associated ILD—like shortness of breath, a dry hacking cough, and fatigue—are common, which means people often brush them off or think that they are signs of aging. Don’t underestimate these common symptoms—they could be signs of something serious, like scleroderma-associated ILD.
Continue Looking for the Right Diagnosis
The common symptoms also make it easy for scleroderma-associated ILD to be misdiagnosed as other conditions, like COPD, asthma, and congestive heart failure. If you’ve been diagnosed with these conditions and have symptoms that won’t get better, it’s time to ask your healthcare provider about scleroderma-associated ILD.
of scleroderma-associated ILD or something else.