Medical Health Encyclopedia

One of the most serious complications of pulmonary fibrosis is interstitial lung disease, which causes a decline in lung function and breathing difficulties. This condition also places the patient at higher risk for lung cancer. (One study suggested that this condition may be due to severe dysfunction in the esophagus that causes patients to aspirate tiny amounts of stomach acid.)

The most important indication of future deterioration appears to be evidence of inflammation in the small airways (called alveolitis), which is detected using a lung test called bronchoalveolar lavage.

Pulmonary Hypertension. Pulmonary hypertension occurs in about half of patients. In this condition, blood pressure in the lungs increases, in some cases to a dangerous level. The primary symptom is shortness of breath, which is often severe.

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It can develop in one of two ways:

• As a complication of pulmonary fibrosis.
• As a direct outcome of the scleroderma process itself. In this case, it is most likely to develop in patients with limited scleroderma after many years.

Pulmonary hypertension can be very serious in the short- and long-term.

• If pulmonary hypertension develops suddenly it can cause respiratory failure, which is life threatening.
• Over time, pulmonary hypertension may cause a condition called cor pulmonale, in which the right side of the heart increases in size. In some cases, this enlargement can lead to heart failure.

Click the icon to see an image of cor pulmonale.

Kidney Complications

Signs of kidney involvement, increased levels of protein in the urine, and mild hypertension are common in scleroderma. As with pulmonary hypertension, the degree of severity depends on whether the situation is acute or chronic.

Slow Progression. The typical course of scleroderma in the kidney is a slow progression that may produce some damage but does not usually require dialysis.