Systemic Sclerosis/Scleroderma
Chronic multisystem disease characterised by progressive fibrosis of organs. Subdivided by pattern of skin and internal organ involvement into:
- Limited cutaneous systemic sclerosis
Skin sclerosis distal to elbows and knees, and prominent vascular malformations. - Diffuse cutaneous systemic sclerosis
Skin thickening proximal to elbows and knees, with early lung fibrosis and ↑ risk of renal and cardiac involvement. - Systemic sclerosis without scleroderma
- Systemic sclerosis with overlap
Any of the above may have features of another systemic rheumatic disease.
Epidemiology and Risk Factors
Pathophysiology
Aetiology
Clinical Manifestations
General:
- Pain
- Fatigue
Respiratory disease:
- Restrictive lung disease
- Pulmonary fibrosis
Cardiac disease:
- Myocardial LV/ failure
- Pulmonary hypertension
- Arrhythmias
Renal failure:
- Renal crisis
Dermatological:
- Raynauds
Diagnostic Approach and DDx
Investigations
Management
Medical
Surgical
Anaesthetic Considerations
- A
- Potential difficult airway
- Small mouth
- ↓ Neck mobility
- Bleeding telangiectasia
- Aspiration risk
- Oesophageal dysmotility
- Potential difficult airway
- B
- Pulmonary fibrosis
- Restrictive lung disease
- C
- Myocardial fibrosis
- Pulmonary hypertension
- Arterial line placement
Beware Raynaud’s and risk of limb ischaemia with small (e.g. radial) arteries.
- E
- Difficult IV access
- G
- Oesophageal dysmotility ↑ risk of TOE injury