Systemic Sclerosis

Question 1

Limited disease?

Answer 1

Skin disease distal to elbows and knees
Raynauds - asymmetrical
Calcinosis
GORD
Sclerodactyly
Telangiectasia

Better prognosis than diffuse disease
10% = Pulmonary HTN = poor survival

Question 2

Diffuse disease?

Answer 2

Widespread skin disease
Rapid progression

Hypertensive renal crisis
Pulmonary fibrosis

–> increased risk of death

Question 3

Treatment of skin disease

Answer 3

Raynauds:

• Covering skin for warmth
• ARBs
• CCBs
• Topical nitrates
• Bosentan and sidenafil

Skin:

• Cyclophosphamide
• MTX

Threatened digits = prostacyclin infusion

Question 4

Treatment of pulmonary HTN

Answer 4

Needs annual screening with echo and lung function tests

Bosentan, ambrisentan, macitentan = non selective endothelin A/B receptor antagonist

Sidenafil = phosphodiaesterase tyoe 5 inhibitor

Iloprost = Prosacyclin agonist

Question 5

Treatment of ILD?

Answer 5

MTX

Question 6

Treatment of scleroderma renal crisis

Answer 6

Captopril

Dose 6hrly doubling until HTN controlled

Increased risk with high dose steroids

Question 7

Most common presenting symptoms of scleroderma?

Answer 7

Raynauds

Question 8

Antibody associated with limited scleroderma?

Answer 8

Anti-centromere antibody

Question 9

Antibody associated with diffuse scleroderma?

Answer 9

Anti-scl70/anti-topoisomerase antibodies

Question 10

Which feature of crest is not included in the diagnostic criteria?

Answer 10

Calcinosis