This article discusses the systemic forms of scleroderma, including manifestations that should alert the GP to the need for investigation and early specialist referral. Advances in drug treatment have resulted in improved symptomatic control and prognosis.
- Features suggesting that Raynaud’s phenomenon is secondary to an underlying illness such as scleroderma include: digital pitting, ulceration or gangrene, oedema of hands and fingers, abnormality of nailfold capillaries and a positive antinuclear antibody. They require rheumatological referral.
- Patients with diffuse systemic sclerosis must be regularly monitored for hypertension, symptoms of breathlessness and progression of skin thickening because early intervention can help prevent significant end-organ damage.
- Oesophageal or symptomatic cardiac involvement should prompt early referral to a gastroenterologist or cardiologist.
- There is increasing evidence for the beneficial effects of immunosuppressive medication, particularly in rapidly progressive diffuse disease.
- It is important to advise maintenance of ambient warmth in those with secondary Raynaud’s, as well as general skin care of digits. This can help with preventing ulceration, gangrene and breakdown of digits. Calcium channel blockers should also be used.