Scleroderma and Interstitial Lung Disease

Interstitial Lung Disease (ILD) is another complication of systemic sclerosis which may affect breathing. Scleroderma-related ILD, or scleroderma-associated Interstitial Lung Disease (SSc-ILD), occurs when there is inflammation and scarring to the lung tissue that supports the alveoli—the air sacs within the lungs. Over time, this scarring, called pulmonary fibrosis, makes it difficult to breathe in and out, and reduces the amount of oxygen that can reach the bloodstream. About 15% of patients with systemic sclerosis have ILD.

Interstitial Lung Disease Symptoms may include:

• Shortness of breath during normal activities
• Difficulty taking deep breaths
• Increased fatigue
• Dry cough that you can’t get rid of
• Feeling dizzy
• Widening of the fingertips
• Muscle and joint pain

ILD Triggers in Scleroderma

There are several factors that are thought to trigger ILD development, or cause it to worsen:

• Inflamed lung tissue
• Scarred lung tissue
• Lung infections
• Occupational exposures to asbestos, coal or grain dust, mold, or silica dust
• Medications such as chemotherapy or immunomodulating drugs, heart medications, and some antibiotics

Treatments for Interstitial Lung Disease (ILD) in Scleroderma

Early detection of ILD in scleroderma is key. The scarring of lung tissue caused by ILD cannot be reversed. There are treatments to improve scleroderma Interstitial Lung Disease symptoms, slow the progression of the disease, and improve patients’ quality of life.

• Those with scleroderma need regular screening for pulmonary lung function.
• Patients may be referred to a pulmonologist for further assessment and treatment.
• Doctors will measure blood oxygen levels at rest and with activity. If they are low, supplementary oxygen may be prescribed.
• Lung transplantation may be suggested to patients with severe ILD with significant scarring.