Systemic Sclerosis

Question 1

What is systemic sclerosis?

Answer 1

Multisystem autoimmune disease - features scleroderma and vascular disease

Question 2

What is the pathophysiology?

Answer 2

Increased fibroblast activity resulting in abnormal growth of connective tissue which leads to vascular damage and fibrosis

Question 3

What are the types of SSc?

Answer 3

Limited

Diffuse

Question 4

What is limited SSc?

Answer 4

Type of SSc characterised by Raynaud’s before any scleroderma

Question 5

What is scleroderma?

Answer 5

Skin thickening

Question 6

What is limited SSc also known as?

Answer 6

CREST syndrome

Question 7

What are the symptoms of limited SSc?

Answer 7

Calcinosis cutis
Raynaud's phenomenon 
Eosophageal dysmobility 
Sclerodactyly 
Telangiectasia

Question 8

What is calcinosis cutis?

Answer 8

Calcium deposits in the skin

Question 9

What is sclerodactyly?

Answer 9

Localised thickening of skin on fingers and toes

Question 10

What is telangiectasia?

Answer 10

Appearance of widened venules on the skin

Question 11

What is diffuse SSc?

Answer 11

Type of SSc characterised by sudden onset of skin involvement

Associated with higher risk of mortality

Question 12

What investigations are done for suspected SSc?

Answer 12

X-rays - show calcinosis

CXR - pulmonary disease
ECG/ECHO - heart problems

Antibodies

• ANA
• anti-centromere
• scl-70
• anti RNA polymerase III

Question 13

How is SSc managed?

Answer 13

Methotrexate + mycophenolate mofetil - reduce skin thickening

CCB/sildenafil - for Raynaud’s

ACEi - prevent hypertensive crisis

Prednisolone - flares