Systemic sclerosis/scleroderma

Question 1

What is systemic sclerosis characterised by?

Answer 1

Increased fibroblast activity causing widespread small vessel/tissue damage

Question 2

What is the old name for limited cutaneous sclerosis?

Answer 2

CREST:
Calcinosis
Raynauds
Esophageal dysmotility
Sclerodactyly
Telangiectasia

Question 3

How does limited cutaneous sclerosis differ from diffuse cutaneous systemic sclerosis?

Answer 3

Limited has a slower onset and progression, and systemic features rarely occur (apart from PHT- happens in 30%)

Question 4

What is diffuse cutaneous systemic sclerosis characterised by?

Answer 4

Rapid progression of skin symptoms (skin changes within a year of Raynauds) and early organ involvement

Question 5

For each system give examples of how systemic sclerosis may manifest:

a) lung
b) MSK
c) cardiac
d) GI

Answer 5

a) fibrosis, PHT
b) Raynaud’s, joint swelling/pain, movement restriction
c) atherosclerosis, IHD, hypertensive cardiomyopathy
d) esophageal dysmotility, constipation, gastroparesis, GORD, oesopaghitis
e) hypertensive renal crisis, failure

Question 6

What are the following autoantibodies associated with in systemic sclerosis?

a) anti-centromere
b) Anti Scl70
c) RNAP

Answer 6

a) limited cutaneous sclerosis
b) lung involvement
c) kidney involvement

Question 7

How would the following symptoms of systemic sclerosis be managed?

a) lung fibrosis
b) PHT
c) Raynaud’s
d) GORD/oesophagitis
e) skin manifestations

Answer 7

a) steroids or immunosuppressants
b) Sildenafil, Iloprost, Bosentan
c) Nifedipine
d) PPI
e) steroids, emollients