Scleroderma

Question 1

Systemic sclerosis

Answer 1

The limited cutaneous form, comprising calcinosis, Raynaud’s, oesophageal dysfunction, sclerodactyly, and telangiectasia (CReSt), is commoner (60% of cases) than the more aggressive diffuse cutaneous form, which has more widespread effects and a high mortality (see E p. 188) (table9.1).

Question 2

Anesthesic considerations

Answer 2

• CVS:Raynaud’s, pericarditis, or myocardial fibrosis. Conduction defects. pulmonary hypertension common.
• pulmonary:fibrosing alveolitis in both forms (40% in diffuseform).
• Renal:may develop renal crisis associated with malignant hypertension.
• GI:oesophageal reflux invariable.
• Airway:may have mouth narrowing and tightened skin around the neck, leading to difficult intubation.
• No consensus on GA versus regional.