When managing scleroderma, it’s important to understand interstitial lung disease (ILD), which is a complication that can affect breathing and lung function.
1. ILD is a common complication of systemic sclerosis.
Approximately 40% of individuals with systemic sclerosis have clinically significant ILD, and 30% of individuals with SSc develop progressive ILD. As with many scleroderma complications, interstitial lung disease progression is variable. Some people may remain stable, others may experience slow progression, and others may experience rapid progression.
2. It causes inflammation and scarring in the lungs.
ILD occurs when there is inflammation and scarring to the lung tissue that supports the alveoli, which are the air sacs within the lungs. Over time, this scarring, called pulmonary fibrosis, makes it difficult to breathe in and out. This reduces the amount of oxygen that can reach the bloodstream.
3. Symptoms can vary—or may not appear at all.
Common symptoms include shortness of breath, fatigue, dry cough, and difficulty taking deep breaths.Some ILD is subclinical, which means the only evidence of this complication is through imaging showing fibrosis in the lungs. In these cases, the person with ILD has normal lung function and capacity and no clinical symptoms.
4. Early screening and monitoring are essential.
People with scleroderma are at the highest risk of developing SSc-ILD within the first few years after their SSc diagnosis. The scarring of lung tissue caused by ILD cannot be reversed. As such, early detection of ILD in scleroderma is key. Doctors may use tests such as pulmonary function tests (PFTs) and high-resolution CT scans (HRCT) to evaluate lung health and monitor for changes over time.
5. Treatment options and research are evolving.
There is a need for more treatment options, and as such, there are currently several clinical trials specifically looking at potential SSc-ILD treatment. This means that down the road, new options may be available to you. If you’re eligible, you could consider participating in a clinical trial where you may have access to try a potential new therapy. As always, consult your doctor when deciding if a clinical trial may be a good option for you.
