Systemic sclerosis

Question 1

What is scleroderma?

Answer 1

Hardening of skin, giving appearance of shiny tight skin

Question 2

What is CREST syndrome?

Answer 2

Calcinosis
Raynaud’s
Oesophageal dysmotility
Sclerodactyly
Telangiectasia

Question 3

What are the signs of limited cutaneous systemic sclerosis?

Answer 3

Raynaud’s
Primarily affects face and distal limbs
CREST syndrome

Question 4

What antibody is seen in limited cutaneous systemic sclerosis?

Answer 4

Anti-centromere antibodies

Question 5

What is the presentation of diffuse systemic sclerosis?

Answer 5

Predominantly affects the trunk and proximal limbs
Scleroderma
CREST syndrome
Systemic complications

Question 6

What antibodies are seen in diffuse systemic sclerosis?

Answer 6

Anti scl-70 antibodies

Question 7

What are the complications of diffuse systemic sclerosis?

Answer 7

Interstitial lung disease
Pulmonary hypertension
Renal disease

Question 8

What investigations are used to diagnose systemic sclerosis?

Answer 8

ACR/EULAR classification criteria
FBC, U&E, LFTs
Antibodies - anti-centromere, anti-scl70

Question 9

What is the non-medical management of systemic sclerosis?

Answer 9

Smoking cessation
Gentle skin stretching
Regular emollients
Avoid cold triggers
Physiotherapy

Question 10

What is the medical management of systemic sclerosis?

Answer 10

Symptomatic mangement
Raynaud’s = nifedipine
Acid reflux - PPI
GI symptoms - metoclopramide
Renal dysfunction - ACEi
Pulmonary HTN - sildenafil