Seminar G - Systemic Sclerosis Flashcards Preview

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Flashcards in Seminar G - Systemic Sclerosis Deck (16):
1

Definition

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

2

EPI

More common in women than men 3:1
UK 18 million/year

3

Peak age

30-50

4

Environmental pollutants

Silica
Epoxy resins
Vinyl chloride
Silicone breast implants

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Pathology

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

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Histologically

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.

7

Collagen accumulation

Type 1 and Type III collagen

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Principal features

Fibrosis
Microvascular damage

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5 year survival

50%

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Classification of Systemic Sclerosis

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

11

CREST

Calcinosis
Raynauds
Esophagus
Sclerodactyly
Telangiectasia

12

FBC

Normocytic
Normochromic anaemia

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ANA

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

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Pulmonary function tests

Restrictive ventilatory defect with reduced transfer factor

15

Echo

Evidence of right-sided dilation consistent with pulmonary hypertension

16

Strategies for Raynauds →

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

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