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Lung Involvement: Lung Fibrosis

The following is a summary about pulmonary (lung) fibrosis, a possible complication of scleroderma.

Much of the information available about pulmonary fibrosis seems scientific and medical in nature and can be hard to follow. Following is a summary prepared directly from the sources cited below.

Another possible pulmonary complication of scleroderma, pulmonary hypertension, is described in the recommendation from the World Health Organization (WHO).

Remember, people with scleroderma are affected differently. Consult your doctor for more information.

Lung Fibrosis

• Approximately 90% of scleroderma (SSC) patients have been found to have pulmonary interstitial fibrotic lung changes (interstitial lung disease-ILD) at postmortem examination
• 8 of 10 (80%) of people with SSC have a problem that may lead to lung scarring
• 4 of 10 (40%) develop significant enough scarring to warrant preventative therapy; 6 of 10 (60%) do not develop significant scarring to warrant therapy
• What happens that causes scarring? Scarring is preceded by inflammation in the lungs (alveolitis). Scarring is caused by an abnormal number of white blood cells accumulating in the lungs
• Result: Lung function deteriorates because lungs are “stiffer” and less able to transfer oxygen to the blood stream
• Inflammation and scarring occur early in the course of scleroderma
• Most significant loss of lung function occurs in the first four to six years of SSC

Symptoms:

• Cough (a cough can be caused by other reasons also)
• Shortness of breath (dyspnea) — most common complaint

Tests:

• Lung X-ray
• Pulmonary Function Test (PFT)
• High Resolution CT (computed tomography) Scan
• Bronchoalveolar Lavage (BAL)
• Lung Biopsy

  (Note: Some physicians recommend baseline tests to measure future tests against.)

Treatments:

• Cyclophosphamide (Cytoxan®)
• azathioprine (Imuran®)
• cyclosporine (Neoral®, Sandimmune®)
• mycophonalate mofetil (Cellcept®)

These drugs have not yet been FDA approved for treating pulmonary fibrosis. They are immunosuppressants and may potentially have serious side effects.

Consult your doctor for more information.

Sources:
Scleroderma Voice, 2002 #4, p. 17 The Scleroderma Lung Study Needs You

Scleroderma Voice, 2003 #3, p. 23 Your Medications: A Guide to Better Understanding

Scleroderma Care and Research, Journal of the Scleroderma Clinical Trials Consortium

Volume 1, Number 1; Premier Issue: Lung Involvement in Scleroderma