Systemic Sclerosis

Question 1

What is systemic sclerosis?

Answer 1

Autoimmune inflammatory progressive multisystem connective tissue disease. Charcterised by small vessel vasculopathy and abnormal collagen deposition leading to fibrosis of the skin and internal organs.

Question 2

Subtypes of systemic sclerosis

Answer 2

• Limited cutaneous systemic sclerosis (CREST)- skin involvement limited distal to knees and elbows. Internal organs still affected
• Diffuse cutaneous systemic sclerosis- diffuse skin involvement past the knees and elbows
• Overlap syndrome

Question 3

Epidemiology of systemic sclerosis

Answer 3

1 in 10,000
4:1 female: male
RFx- silicone breast implants, previous chemotherapy (esp bleomycin), silica dust, exposure to gadolinium

Question 4

Extra-cutaneous conditions associated with systemic sclerosis

Answer 4

• CKD
• Pulmonary fibrosis
• Pulmonary hypertension
• Myocardial ischaemia
• OSA
• Arrhythmias
• CVA
• Chronic pain from peripheral neuropathies and skin lesions

Question 5

Pathophysiology of systemic sclerosis

Answer 5

An unknown trigger in a susceptible individual leads to…
- Endothelial cells proliferate and cause vasoconstriction leading to localised tissue necrosis and large vessel disease (pulm HTN, renal crisis)
- Fibroblasts produce aberrant collagen and lead to tissue fibrosis and loss of elasticity
- Inflammatory mediators ET1, PDGF released and anti-centromere antibodies produced

Question 6

CFx of systemic sclerosis

Answer 6

Cutaneous:
- sclerodactyly
- Calcinosis
- Widespread telangiectasia
- Puffy hands
- Raynaud’s

Organs-
- PAH
- CKD
- Reflux

Question 7

Pharmacological treatment for systemic sclerosis

Answer 7

1. Immunomodulation: steroids, rituximab, mycophenolate, tocilizumab
2. ILD antifibrotics: nintedanib
3. Vasodilators: Ca channel blockers, phosphodiesterase inhibitors, prostacyclin analogues
4. General cardiac disease modulators: ACEi, diuretics
5. General GI disease modulators: PPI, antacid

Question 8

Anaesthetic Considerations for Systemic Sclerosis

Answer 8

Airway
- Can have limited mouth opening and neck movement may need ATI
- OSA common

Respiratory
- Interstitial lung disease and fibrosis, restrictive defects
- Difficult ventilation
- Weakness may need post-op ventilation

Cardiovascular
- Heart blocks common
- Risk of MI
- Risk of rt heart failure
- Can have pericarditis

CNS
- Seizures and headaches are common
- Often chronic pain and peripheral neuropathies

MSK
- Difficult IV access
- Difficult pulse oximetry
- Muscle weakness common in late disease
- Pressure sore
- Raynauds- potentially severe pain on IV propofol

GI
- High aspiration risk
- Malabsorption and malnutrition common

Renal
- High risk of systemic sclerosis renal injury
- AKI

Haematological
- High risk of VTE
- Some overlap with anti-phospholipid syndrome
- Anaemia of chronic disease