The majority of patients with scleroderma lung disease do not need treatment, especially if the lung involvement is limited and remains stable over time.
Treatment may be needed if the lung disease is significant (is causing a lot of symptoms or is showing up as a major limitation in lung function testing) or if it is getting progressively worse. When there is fibrosis that needs treatment, medications are used that reduce the inflammation that causes the fibrosis. However, treatments that are used are not able to remove fibrosis that has already occurred. For this reason, the main aim of treatment for the lungs is to prevent worsening in breathing symptoms and lung function; thus the first aim of treatment is to stabilise lung function and prevent further progression. In some individuals, the treatment of the inflammation in the lungs leads to noticeable improvements in symptoms and lung function. However, as the primary aim of therapy is to prevent fibrosis getting worse, stabilisation of symptoms and lung function alone is evidence of response to treatment and should not be viewed as a treatment failure.
Where treatment for scleroderma-associated lung fibrosis is needed, a number of drugs may be used. Many of these are also used purely for the skin or joint problems caused by scleroderma. Other treatments are more specifically designed to treat the lung disease.
The most commonly used treatment for the lungs is a combination of low-dose steroids (usually 10 mg once daily) together with an immunosuppressant drug. The immunosuppressants that are most frequently used for scleroderma, and both of which come in tablet form, are azathioprine and mycophenolate. These immunosuppressants reduce the overactivity of the immune system which plays a crucial part in the development of irreversible lung scarring. In individuals with extensive or rapidly worsening lung fibrosis, intravenous treatment (by drip infusion) with cyclophosphamide may be used. Cyclophosphamide is also an immunosuppressant but when used intravenously it acts more quickly than other immunosuppressant medications.
Treatment of lung fibrosis needs to be continued for as long as there is evidence of ongoing inflammatory activity due to scleroderma. For most individuals with scleroderma and lung fibrosis, however, it is usually possible to gradually reduce and stop treatment once the lung disease is no longer active. It is important to realise that this process of waiting for inflammatory activity to subside and then gradually reducing and stopping treatment may take several years.
Meet the ILD team
Clinical nurse specialist
Interstitial lung disease unit
Lind ward, Fulham wing/South block, Royal Brompton Hospital
Telephone: +44(0)20 7351 8327
Fax: +44(0)20 7351 8918