SSc

Question 1

Distinguish between Systemic Sclerosis and scleroderma.

Answer 1

• diffuse fibrosis (sclerosis) of connective tissues
• degenerative changes

•	vascular abnormalities
o	skin (if only skin → scleroderma) 
o	articular structures 
o	internal organs

Question 2

Write brief notes on the 3 (three) main pathological processes identified in SSc.

Answer 2

•	vasculitis
•	marked fibrosis 
•	increased fibroblastic activity results in:
o	 increased collagen synthesis
o	increased collagen turnover 

• A variety of immunologic abnormalities

Question 3

Describe the clinical manifestations with which SSc typically presents.

Answer 3

• Reynauds
• insidious swelling of acral portions of extremities
• gradual thickening and induration of skin of fingers
• polyarthralgia (= prominent early symptom)

Question 4

How is the skin affected in SSc?

Answer 4

• SSc usually first manifests in the skin of the face and fingers

• The progression of the disease in the skin is as follows:
o skin becomes oedematous
o observe vasculitis and petechial haemorrhages
o progressive fibrosis involving the dermis and subcutaneous tissue

• As a result of these changes we observe:
o epidermal: thinning, shininess, tautness
o epidermal: hyperpigmentation
o skin adnexal atrophy
o telangiectasis
o trophic ulceration
o dystrophic calcification

• Final result:
o hands: claw - like contractions
o face: restricted movements

Question 5

How are the kidneys affected in SSc?

Answer 5

• immune complexes deposition in the glomeruli which results in:
o basement membrane thickening
o mesangial hypercellularity

• intimal fibrosis of small arterioles which results in:
o glomerular ischaemia → decreased GFR → renal failure

Question 6

How is the gastrointestinal system affected in SSc?

Answer 6

• Oesophagus and small intestine are most affected.

• GIT involvement results in:
o dysphagia and peptic oesophagitis
o poor peristalsis
o malabsorption

Question 7

What is the CREST syndrome?

Answer 7

relatively benign form off SSc

Question 8

What does CREST stand for?

Answer 8

o	Calcinosis
o	Raynaud’s phenomenon
o	Esophageal disease
o	Sclerodactyly
o	Telangiectasis

Question 9

Discuss the diagnosis and prognosis of SSc.

Answer 9

Diagnosis: Typical clinical manifestations are unmistakable

Prognosis
• SSc is usually a chronic slowly progressive condition.
• Prognosis is poor with early involvement of the cardiac, pulmonary and renal systems: