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Lifestyle Modifications and Quality of Life for Scleroderma
Pulmonary Involvement in Scleroderma
Pulmonary (lung) involvement is common in patients with systemic scleroderma and any signs of problems must be addressed immediately. Lung infections must be treated as soon as possible to prevent complications. Patients should notify their doctor immediately if they notice:
- Shortness of breath
- Fatigue
- Difficulty breathing
Pulmonary fibrosis may be responsive to corticosteroids or other immunosuppressive agents (e.g., cyclophosphamide) but effective treatment for this serious complication remains elusive. Patients who develop alveolitis (inflammation in the small air sacs of the lungs) may also benefit by treatment with cyclophosphamide.
Pulmonary hypertension is a serious complication in patients with systemic scleroderma and carries a poor prognosis if it does not respond to medication. So far there is no standard drug regimen used to effectively treat pulmonary hypertension. Prostacyclins, such as Iloprost, are potent vasodilators and are currently the most widely used drugs for pulmonary hypertension.
Bosentan (Tracleer), an endothelial receptor antagonist drug, was approved by the FDA for the treatment of pulmonary hypertension in 2001. This drug blocks an enzyme called endothelin which causes the constriction of blood vessels.
