Systemic Sclerosis (Scleroderma)

Question 1

Systemic sclerosis is a condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues. It is four times more common in females.

What are the names of the three patterns of disease?

Answer 1

1. Limited cutaneous systemic sclerosis
2. Diffuse cutaneous systemic sclerosis
3. Scleroderma

Question 2

What is limited cutaneous systemic sclerosis?

Answer 2

• Raynaud’s may be first sign
• Scleroderma affects face + distal limbs predominantly
• Associated w/ anti-centromere antibodies
• A subtype of limited systemic sclerosis is CREST syndrome

Question 3

What is CREST syndrome?

Answer 3

• Calcinosis
• Raynaud’s phenomenon
• Esophageal dysmotility
• Sclerodactyly → thickening + tightness of skin of fingers
• Telangectasia

Question 4

What are features of diffuse cutaneous systemic sclerosis?

Answer 4

• Scleroderma affects trunk + proximal limbs predominantly
• Associated w/ SCL-70 antibodies
• Most common cause of death is now resp involvement, seen in around 80%: interstitial lung disease + pulmonary arterial hypertension
• Other complications → renal disease + hypertension
• Poor prognosis

Question 5

What are features of scleroderma (without internal organ involvement)?

Answer 5

• Tightening + fibrosis of skin
• May manifest as plaques (morphoea) or linea

Question 6

What antibodies are present in scleroderma?

Answer 6

• ANA positive in 90%
• RF positive in 30%
• anti-scl-70 associated w/ diffuse cutaneous systemic sclerosis
• anti-centromere antibodies associated w/ limited cutaneous systemic sclerosis