Medical Health Encyclopedia

Many patients with systemic scleroderma experience a plateau in which the condition stabilizes, which is followed with a period of improvement and skin softening. No one knows why this occurs and it can occur without any association to treatment. In one study, patients with systemic scleroderma who experienced such improvements also had better survival rates (80% at 10 years) than those whose skin did not improve (60% 10-year survival rate).

Lung Complications

Lung problems are usually the most serious complications of systemic scleroderma. They are now the leading cause of death in scleroderma patients. Two major lung conditions associated with scleroderma, pulmonary fibrosis and pulmonary hypertension, can occur either together or independently.

Pulmonary hypertension is the narrowing of the pulmonary arterioles within the lung. The narrowing of the arteries creates resistance and an increased work load for the heart. The heart becomes enlarged from pumping blood against the resistance. Some symptoms include chest pain, weakness, shortness of breath, and fatigue. The goal of treatment is control of the symptoms, although the disease usually develops into congestive heart failure.

Text Continues Below

---

Pulmonary Fibrosis. Scleroderma in the lung causes scarring (pulmonary fibrosis). Pulmonary fibrosis occurs in up to 80% of patients, although the progression is very slow and patients respond with a wide range of symptoms:

• Some patients may not even experience symptoms.
• When it progresses, patients develop a dry cough, shortness of breath, and reduced exercise capacity.
• Severe pulmonary fibrosis occurs in about 16% of patients with diffuse scleroderma. About half of these patients experience the most profound changes within the first three years. In such cases, lung function declines rapidly over that period and then slows down.