Management

Treatment is essentially symptomatic. Hands and feet should be kept warm and regular chiropody offered. Any infection, dysphagia or hypertension should be treated. Different treatment regimes have been used in the management of this disease:

• ACE inhibitors - management and prevention of renal failure
• penicillamine - has been used in an attempt to halt the fibrotic process
• systemic steroids combined with cyclophosphamide - has been used in the management of respiratory disease
• H2 blockers / proton pump inhibitors for symptoms of reflux

Raynaud's phenomenon may be successfully managed.

The disease may be mild or severe. The mean 5-year survival is about 70% in the absence of renal disease. Increasing levels of plasma renin may herald rapid deterioration.