Systemic Sclerosis

Question 1

What is systemic sclerosis? And it’s pathogenesis?

What are the 2 main subtypes?

Answer 1

A multi system autoimmune disease, also known as scleroderma

Increased fibroblast activity results in abnormal growth of connective tissue leading to vascular damage and fibrosis

Limited SSc
Diffuse SSc

Question 2

What are the common symptoms and signs of limited SSc?

Answer 2

CREST
Calcinosis Cutis (calcium deposits in skin)
Raynaud's phenomenon 
oEsophageal dysmotility
Sclerodactyly
Telangiectasia

Develop pulmonary arterial HTN after many years of symptoms.
Often present with Raynaud’s syndrome before scleroderma

Question 3

What are the common symptoms and signs of diffuse SSc?

Answer 3

Sudden onset of skin involvement proximal to elbows and knees. Less common, has a higher mortality risk

Question 4

What is seen on x-ray in Ssc?

Answer 4

Calcinosis

Question 5

What is seen on CXR, HRCT and PFTs in SSc?

Answer 5

Pulmonary disease

Question 6

What is seen on ECG and ECHO in SSc?

Answer 6

PA (pulmonary arterial) hypertension, heart failure, myocarditis and arrhythmias

Question 7

What is the most commonly positive antibody in SSc?

Which is associated with limited and which two with diffuse?

Answer 7

ANA (anti-nuclear antibody)

Anti-centromere antibody is strongly associated with limited SSc

Scl-70 and anti-RNA polymerase III antibodies are strongly associated with diffuse SSc

Question 8

What is the treatment for SSc?

Answer 8

Treat each part of SSc

• Psychological support
• Calcium antagonists/sildenafil/iloprost infusion for Raynaud’s
• Methotrexate and mycophenolate mofetil - reduce skin thickening?
• ACEi to prevent HTN
• Prednisolone short courses for flares
• PPI for GI symptoms

Question 9

Which gender is SSc more common in?

Answer 9

5 times more common in women

Question 10

What is scleroderma renal crisis?

Answer 10

Serious condition - accelerated HTN - leads to renal failure if not treated quickly. Usually seen in diffuse SSc