Seminar G - Systemic Sclerosis

Question 1

Definition

Answer 1

A group of disorders characterised by the development of hardened, sclerotic skin.

Question 2

EPI

Answer 2

More common in women than men 3:1

UK 18 million/year

Question 3

Peak age

Answer 3

30-50

Question 4

Environmental pollutants

Answer 4

Silica
Epoxy resins
Vinyl chloride
Silicone breast implants

Question 5

Pathology

Answer 5

Over-production of connective tissue – collagen, with fibrosis and widespread microvascular damage (accounts for raynauds and organ damage)

Question 6

Histologically

Answer 6

Narrowing of the digital arteries with intimal hyperplasia and endothelial damage
Results in platelet activation and the release of potent vasoconstrictors such as 5-HT anf thromboxane A2.

Question 7

Collagen accumulation

Answer 7

Type 1 and Type III collagen

Question 8

Principal features

Answer 8

Fibrosis

Microvascular damage

Question 9

5 year survival

Answer 9

50%

Question 10

Classification of Systemic Sclerosis

Answer 10

• Limited cutaneous systemic sclerosis – CREST
• Diffuse cutaneous systemic sclerosis
• Environmentally induced scleroderma
• Mixed Connective Tissue Disease/Overlap syndromes

Question 11

CREST

Answer 11

Calcinosis
Raynauds
Esophagus
Sclerodactyly
Telangiectasia

Question 12

FBC

Answer 12

Normocytic

Normochromic anaemia

Question 13

ANA

Answer 13

Positive
• Anti-centromere
• Anti-Scl-70 antibodies

Question 14

Pulmonary function tests

Answer 14

Restrictive ventilatory defect with reduced transfer factor

Question 15

Echo

Answer 15

Evidence of right-sided dilation consistent with pulmonary hypertension

Question 16

Strategies for Raynauds →

Answer 16

1. Stop Smoking
2. Keep hands warm
3. Drugs e.g. nifedipine, ACE inhibitors
4. Avoid Beta Blockers
5. IV prostacyclin if severe