Systemic Sclerosis

Question 1

what are the 2 types of systemic sclerosis

Answer 1

1) Limited cutaneous systemic sclerosis

2) Diffuse cutaneous systemic sclerosis

Question 2

features of limited cutaneous systemic sclerosis

Answer 2

1) Calcinosis
2) Raynaud’s
3) Oesophageal dysmotility
4) Sclerodactyly
5) Telangiectasia

Question 3

what other features might you see in diffuse cutaenous systemic sclerosis

Answer 3

• This has the features of CREST but also affects the internal organs causing:
  1) CVD – HTN, CAD
  2) Lung – pulmonary HTN, PF
  3) Kidney – glomerulonephritis, scleroderma renal crisis

Question 4

define scleroderma

Answer 4

1) Scleroderma  hardening of the skin (shiny, tight skin without fold, most notable on the hands and face)

Question 5

define sclerodactyly

Answer 5

2) Sclerodactyly  as skin tightens, reduces range of motion hence function, the fat pads on the fingers are lost and the skin can ulcerate and break.

Question 6

what antibodies would you test for systemic sclerosis

Answer 6

1) ANA are positive in most patients
2) Anti-centromere antibodies (limited cutaneous systemic sclerosis)
3) Anti-Scl-70 (diffuse cutaneous systemic sclerosis)

Question 7

what would you investigate in systemic sclerosis if the patients had Raynaud’s to exclude primary raynaud’s?

Answer 7

• Systemic sclerosis  avascular areas, abnormal capillaries, micro-haemorrhage.
• Useful in patients with Raynaud’s to exclude systemic sclerosis as if isolated Raynaud’s then their nail fold will be normal.

Question 8

non-medical management of systemic sclerosis ?

Answer 8

1) Avoid smoking
2) Gentle skin stretching to maintain motion
3) Regular emollients
4) Avoid cold triggers for Raynaud’s
5) Physio
6) Occupational therapy

Question 9

medical mx of systemic sclerosis?

Answer 9

1) Nifedipine to treat Raynaud’s
2) PPIs and metoclopramide for GI
3) Analgesia for joint pain
4) Antibiotics for skin infections
5) Antihypertensives for HTN
6) Supportive management for PF