Lung Problems From Scleroderma (Systemic Sclerosis)

Interstitial Lung Disease and Pulmonary Hypertension

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Lung disease is a serious potential health condition that can develop in people with systemic sclerosis (scleroderma). Interstitial lung disease occurs in most, but not all people with the condition. It is the leading cause of death among people with systemic sclerosis.

Pulmonary hypertension is another less common lung problem in systemic sclerosis. It is the second leading cause of death in people with the condition.

Systemic Sclerosis

In some forms of scleroderma, internal organs such as the lungs are not affected. Therefore, this article uses the more specific term “systemic sclerosis” instead.

Senior woman suffering from chest pain in bed

Milko / Getty Images

Lung Issues From Systemic Sclerosis

People with systemic sclerosis can develop specific problems with their lungs.

Interstitial Lung Disease Symptoms

Cough and shortness of breath are common symptoms in people with interstitial lung disease from systemic sclerosis. Tiredness and weakness can also occur.

However, not everyone with interstitial lung disease from systemic sclerosis has symptoms, especially if their condition is mild.

When the disease is severe, respiratory failure and death are possible.  

Pulmonary Hypertension Symptoms

Systemic sclerosis can also cause elevated blood pressure in the blood vessels of the lungs (pulmonary hypertension). This often happens along with interstitial lung disease. However, some people with systemic sclerosis may have one condition and not the other.

People who have both interstitial lung disease and pulmonary hypertension tend to develop worse disease symptoms and do not live as long as people who have just one condition. 

Pulmonary hypertension does not always cause symptoms at first. However, over time, symptoms like shortness of breath may develop and get worse.

Pulmonary hypertension increases the work that your heart must do to pump blood through the vessels of the lungs. Therefore, a person may experience symptoms like chest pain and swelling of the extremities. If severe, this can eventually lead to life-threatening symptoms. 

Pulmonary Hypertension

High blood pressure (hypertension) is not the same condition as pulmonary hypertension.

When you hear that a person has high blood pressure, it means that the pressure is high in the blood vessels that go out to the rest of the body, not in the vessels going to the lungs, as is the case in pulmonary hypertension.

Pulmonary hypertension also creates a different set of health issues than high blood pressure.

Causes

Systemic sclerosis is an autoimmune disease. Genetics and environmental factors likely play a role in triggering the disease, but much about the disease is unknown.

Although researchers are not sure exactly what causes systemic sclerosis, some parts of the immune system appear to be overactivated in people who have the condition. The body starts reacting to parts of itself as if they were foreign invaders, which sets off inflammation and other immune system issues.

In systemic sclerosis, part of the problem is the excess formation of scar tissue (fibrosis). Although this can be a normal part of the immune response in some circumstances, in people with systemic sclerosis the fibrosis gets out of control.

Excess scar tissue can lead to problems all over the body. For example, it can cause hardened, thickened skin.

Systemic sclerosis can also affect the internal organs. When fibrosis affects the gastrointestinal tract, it can cause issues like reflux. Fibrosis of other internal organs, such as the kidneys, can also cause health complications.

In many people with systemic sclerosis, the lungs are also affected. Excess scar tissue in the lungs leads to interstitial lung disease, which causes the lung tissue to be more constricted than usual.

Excess scar tissue can also damage the blood vessels in the lungs and lead to pulmonary hypertension.

Risk Factors 

Not everyone with systemic sclerosis develops interstitial lung disease. However, certain factors can increase a person’s risk.

Factors that might make it more likely that a person will get interstitial lung disease include:

  • Having the diffuse cutaneous form of systemic sclerosis
  • Being African American
  • Being of older age when systemic sclerosis symptoms started

Antibodies

People who have a certain antibody—anti-Scl-70 (or anti-topoisomerase I)—are more likely to get interstitial lung disease.

Not having another antibody—anti-centromere—increases a person’s risk for interstitial lung disease.

Symptoms and Risks

People are also more likely to develop interstitial lung disease if they have not had other symptoms for a long time. If you’ve had systemic sclerosis symptoms for several years already, this lowers the risk that you’ll ever experience problems from lung disease.

Other Types of Interstitial Lung Diseases

Other types of autoimmune diseases can cause symptoms from interstitial lung disease. These diseases also can affect connective tissue (a sort of filler tissue in the body). These conditions all have some overlapping causes.

For example, interstitial lung disease is sometimes (but not always) found in people with the following conditions:

Diagnosis

People with interstitial lung disease from systemic sclerosis are most often diagnosed after their initial diagnosis with systemic sclerosis. Everyone with systemic sclerosis should be screened for the possibility of lung disease. 

The screening will include a careful medical history and physical exam. Your doctor will assess your basic lung function—for example, by measuring how far you can walk in six minutes.  

The assessment should also include a complete set of pulmonary function tests, which show how well you breathe and how well your lungs are working overall.

You should also have a lung imaging test called high-resolution computed tomography (HRCT), which is a very detailed CT scan.

You might need to have an echocardiogram, which will give your doctor an idea of whether you might have pulmonary hypertension. It will also show whether pulmonary hypertension might be putting strain on your heart.

Sometimes, the symptoms from the interstitial lung disease are noticed before those from systemic sclerosis (which is later identified as the underlying cause of the lung disease).

If new symptoms emerge, such as Raynaud’s syndrome or spider veins (telangiectasia), it can point to systemic sclerosis as a diagnosis.

Monitoring for Interstitial Lung Disease in Systemic Sclerosis

Even if you do not have interstitial lung disease at your initial diagnosis of systemic sclerosis, your doctor will want to monitor you for it.

Screening is especially important in the first few years after your symptoms appear because that’s when it’s most likely that interstitial lung disease will develop. During this time, you may need to have certain screening tests, like pulmonary function tests, every four to six months.

By getting regular screenings, your doctor is more likely to catch lung problems early on. Early diagnosis is important because the sooner lung problems are treated, the more likely it is that lung function can be preserved for a longer time.

Treatment

The treatment options for interstitial lung disease from systemic sclerosis are limited. Treatments like supplemental oxygen may help some people, while other treatments may slow—but not cure— the disease.

Immunosuppressants

Corticosteroids are commonly prescribed as the initial therapy. If symptoms do not get better, additional treatments to tone down the immune system are necessary.

The most commonly prescribed medications are Cytoxan (cyclophosphamide) and CellCept (mycophenolate mofetil). Both are heavy-duty immunosuppressive drugs that carry the risk of certain side effects, such as potentially increasing the chance of severe infections. 

Recently, another type of therapy, nintedanib, was approved by the Food and Drug Administration (FDA). This treatment helps slow the scarring in the lungs, which may help keep a person’s lung function from declining as quickly.

Other Prescription Medications

Certain treatments may help with pulmonary hypertension that is caused by systemic sclerosis. However, it’s not clear if these medications are effective specifically in people with pulmonary hypertension from systemic sclerosis. 

Doctors sometimes treat pulmonary hypertension from different causes with treatments, such as:

  • Norvasc (amlodipine)
  • Tracleer (bosentan)
  • Viagra (sildenafil)

Stem Cells

Some people with systemic sclerosis have successfully received stem cell transplantation as a treatment for severe lung disease. However, this treatment carries a significant risk of serious side effects, such as infections.

Stem cell treatment for people with lung disease from systemic sclerosis is only available at a small number of medical centers.

Lung Transplant

Lung transplant is also a theoretical option, but it is not done often. People with systemic sclerosis typically have other serious health issues, and only a limited number of lung transplants are available.

People with systemic sclerosis might be less likely to be selected to receive a set of donated lungs because doctors may worry that people with the condition will not be able to handle the stress of surgery and follow-up immunosuppressive treatment.

Research

Researchers are actively looking for better treatments for lung disease from systemic sclerosis which will more effectively treat the disease with less significant risks and potential side effects. Several therapies are currently being investigated.

Prognosis

The prognosis for people with interstitial lung disease from systemic sclerosis is usually not good. People who have interstitial lung disease along with systemic sclerosis are much more likely to die from disease complications than people who do not have lung disease.

Around 80% to 90% are alive five years after being diagnosed with interstitial lung disease from systemic sclerosis. The rate is lower in people who have both interstitial lung disease and pulmonary hypertension.

However, some people with lung disease stabilize and live much longer. People who are treated early on for their lung disease may also do better than people who are treated later or who are not receiving treatment.

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