Scleroderma (Systemic Sclerosis) (3)

Question 1

What does Scleroderma mean?

What is Systemic Sclerosis?
→ What are the 2 patterns of it?

Answer 1

➊ Hardening of skin

➋ Autoimmune inflammatory and fibrotic connective tissue disease
→ • Limited cutaneous systemic sclerosis
• Diffuse cutaneous systemic sclerosis

Question 2

Limited cutaneous systemic sclerosis:
What are the features of it?

Which antibody is diagnostic for it?

Answer 2

➊ CREST:
• Calcinosis
• Raynaud’s phenomenon
• Oesophageal dysmotility (Dysphagia, GORD)
• Sclerodactyly
• Telangiectasia

➋ Anti-centromere

Question 3

Diffuse cutaneous systemic sclerosis:
What are the features of it?

Which antibody is diagnostic for it?

Answer 3

➊ Those of CREST plus:
• CVS problems – HTN, Coronary artery disease
• Lung problems – Pulmonary HTN, Fibrosis
• Kidney problems – Glomerulonephritis, Scleroderma renal crisis

➋ Anti-Scl-70