Systemic sclerosis

Question 1

define Systemic sclerosis ?

Answer 1

: rare connective tissue disease characterised by widespread small blood vessel damage and fibrosis in skin

Question 2

what conditions are encompassed in systemic sclerosis

Answer 2

o Pre-Scleroderma
o Diffuse Cutaneous Systemic Sclerosis
o Limited Cutaneous Systemic Sclerosis

Question 3

what are the features of Diffuse Cutaneous Systemic Sclerosis ?

Answer 3

o	Mainly affects the trunk and proximal limbs 
o	Respiratory involvement 
o	Renal disease 
o	Hypertension  
o	Associated with scl-70 antibodies

Question 4

what are the features of limited Cutaneous Systemic Sclerosis ?

Answer 4

o Affects face and distal limbs first
o Associated with anti-centromere antibodies
o Previously known as CREST Syndrome

Question 5

what does CREST stand for?

Answer 5

• Calcinosis
• Raynaud’s phenomenon
• (O)esophageal dysmotility
• Sclerodactyly
• Telangiectasia

Question 6

epidemiology of systemic sclerosis?

Answer 6

more common in females

Question 7

presenting symptoms of systemic sclerosis?

Answer 7

• Skin - Raynaud’s phenomenon
• Dysphagia
o thickened swollen painful fingers
o Microstomia

Question 8

which antibodies are associated with diffuse cutaneous systemic sclerosis ?

Answer 8

o Anti-scl-70 antibodies

Question 9

which antibodies are associated with

Answer 9

o Anti-centromere antibodies