Systemic Sclerosis (SSC)

Question 1

pathogenesis

Answer 1

excessive collagen deposition - skin and internal organ changes 
vasomotor disturbances (raynauds) 
fibrosis 
subsequent atrophy of the skin and subcutaneous tissue 
3 phases of cutaneous symptoms 
- odematous 
- indurative 
- atrophic

Question 2

prognosis

Answer 2

renal and lung changes responsible for death

pulmonary hypertension leads to 12% of systemic sclerosis-related deaths

Question 3

How does it present?

Answer 3

Raynaud’s phenomenon
skin becomes tight and thickened
skin of the face affected
- pinching of the skin of the nose (beaking)
- tightening of the skin around the mouth
telangiectasia
calcinosis - subcutaneous deposits of calcium may be seen in the digits
major features - centrally located skin sclerosis (arm, face, neck)
minor features - sclerodactyly
and atrophy of the fingertips and bilateral lung fibrosis

Question 4

what is the presentation of organ involvement in systemic sclerosis?

Answer 4

lung - pulmonary hypertension, pulmonary fibrosis
accelerated hypertension - leading to renal crisis
gut involvement - dysphagia, malabsorption, bacterial overgrowth of the small bowel
inflammatory arthritis and myositis

Question 5

how is it classified?

Answer 5

Limited (aka CREST)
- skin involved confined to face, hands, forearms and feet 
- organ involvement occur later 
- anti-centromere
Diffuse 
- skin changes develop more rapidly, can involve the trunk 
- early significant organ involvement 
- anti-Scl-70

Question 6

how is it investigated?

Answer 6

immunology 
regular organ screening
- pulmonary function test 
- echo 
- monitor renal function

Question 7

how is it diagnosed?

Answer 7

patient needs 1 major feature and 2 minor features
major features
- centrally located skin sclerosis
- arms, face, neck
minor features
- sclerodactyly and atrophy of the fingertips
- bilateral lung fibrosis

Question 8

how is it managed?

Answer 8

tailored to specific issues
Raynauds/digital ulcers - CCBs (calcium channel blockers) Iloprost, Bosentan
Renal involvement - ACEi
GI involvement - PPI for reflux
ILD - immunosuppression - usually cyclophoysphamide