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.
Verywell / Laura Porter
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.
When the disease is severe,respiratory failureand death are possible.
This often happens along with interstitial lung disease.
However, some people with systemic sclerosis may have one condition and not the other.
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.
Pulmonary hypertension also creates a different set of health issues than high blood pressure.
Causes
Systemic sclerosis is anautoimmune disease.
In systemic sclerosis, part of the problem is the excess formation of scar tissue (fibrosis).
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 thekidneys, can also cause health complications.
In many people with systemic sclerosis, the lungs are also affected.
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 persons risk.
Not having another antibodyanti-centromereincreases a persons risk for 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.
Everyone with systemic sclerosis should be screened for the possibility of lung disease.
The screening will include a careful medical history and physical exam.
It will also show whether pulmonary hypertension might be putting strain on your heart.
By getting regular screenings, your doctor is more likely to catch lung problems early on.
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 slowbut not cure the disease.
Immunosuppressants
Corticosteroidsare commonly prescribed as the initial therapy.
If symptoms do not get better, additional treatments to tone down the immune system are necessary.
Other Prescription Medications
Certain treatments may help with pulmonary hypertension that is triggered by systemic sclerosis.
However, its not clear if these medications are effective specifically in people with pulmonary hypertension from systemic sclerosis.
However, this treatment carries a significant risk of serious side effects, such as infections.
Lung Transplant
Lung transplant is also a theoretical option, but it is not done often.
Several therapies are currently being investigated.
Prognosis
The prognosis for people with interstitial lung disease from systemic sclerosis is usually not good.
However, some people with lung disease stabilize and live much longer.
Short- and long-term outcomes.Ann Am Thorac Soc.
