Systemic sclerosis

Question 1

What is systemic sclerosis? And what is the pathophysiology?

Answer 1

Multisystem autoimmune disease.
Previously known as scleroderma.
Increased fibroblast activity resulting in abnormal
growth of connective tissue which leads to
vascular damage and fibrosis.

Question 2

What are the types of systemic sclerosis?

Answer 2

Limited (CREST)

Diffuse

Question 3

What is CREST syndrome? How does it present?

Answer 3

Another name for limited scleroderma.
C alcinosis Cutis
R aynaud’s phenomenon
E osophageal dysmotility
S clerodactyly
T elangiectasia
(Many years of Raynaud's first, PA hypertension develops after about 10 yrs)

Question 4

How is diffuse scleroderma characterised?

Answer 4

Characterised by sudden onset of skin involvement,

and is proximal to the elbows and knees.

Question 5

What antibody tests are done in systemic sclerosis?

Answer 5

Positive ANA in 90% of patients

Anti-centromere antibody strongly associated with
limited SSc.

Scl-70 (topisomerase) and anti RNA polymerase III
antibodies strongly associated with diffuse SSc.

Question 6

How is SSc managed?

Answer 6

No cure- psychological support
• Calcium antagonist/sildenafil/iloprost infusion- Raynaud’s symptoms
•Methotrexate and mycophenolae mofetil-
reduce skin thickening
• ACEi- prevent hypertensive crisis & reduce mortality from renal failure
• Short courses of prednisolone for flares
• PPI- GI symptoms