Systemic sclerosis

Question 1

What is the old name for systemic sclerosis?

Answer 1

Scleroderma

Question 2

What is systemic sclerosis?

Answer 2

A condition in which there is thickening and fibrosis of the skin, vasculature and other systemic tissue

Question 3

Who is most likely to have systemic sclerosis?

Answer 3

Females

Question 4

What disease is commonly secondary to systemic sclerosis?

Answer 4

Raynaud’s disease, with 90% of SS patients presenting also with Raynaud’s

Question 5

What are the 2 varieties of systemic sclerosis?

Answer 5

Diffuse cutaneous systemic sclerosis
Limited cutaneous systemic sclerosis

Question 6

Describe briefly the pathophysiology of systemic sclerosis

Answer 6

Factors cause production of autoantibodies =>
Endothelial activation, leukocyte attraction and vascular occlusion =>
Macrophage activation =>
T and B cell activation =>
Cytokine release
Conversion of fibroblasts and fibrocytes into myofibroblasts =>
Laying down of scar tissue and fibrosis

Question 7

Describe the difference between DCSS and LCSS

Answer 7

DCSS will cause skin thickening and scarring on extremities AND upper limbs, trunk and face

LCSS will cause skin thickening and scarring only below the knees and elbows with limited facial involvement

Question 8

What are the main presentations of LCSS?

Answer 8

CREST:
C - Calcinosis
R - Raynaud’s phenomenon
E - Oesophageal dysfunction
S - Sclerodactyly
T - Telangiectasia

Question 9

What is sclerodactyly?

Answer 9

Thickening and tightening of the skin on the fingers and hands

Question 10

What is telangiectasia?

Answer 10

Dilatation of capilaries causing red marks on the skin

Question 11

As well as CREST symptoms, what are some further complications of DCSS?

Answer 11

Pulmonary fibrosis
Pulmonary hypertension
Sclerodermal renal crisis

Question 12

How does tissue sclerosis cause pulmonary hypertension?

Answer 12

Thickening of the lung vasculature and alveolar walls causes an increase in blood pressure in the pulmonary artery, and thus increased pressure in the right side of the heart

Question 13

What tests can be used to aid diagnosis of systemic sclerosis?

Answer 13

History
Examination
Pulmonary function testing
Renal function testing
Immunological testing

Question 14

What antibodies can be found in patients with systemic sclerosis?

Answer 14

Anti-centromere antibodies (20-30%)
Anti-topoisomerase antibodies (15-20%)
Anti-RNA polymerase (20%)

Question 15

When are anti-centromere antibodies often found in systemic sclerosis?

Answer 15

In LCSS, Crest syndrome and pulmonary hypertension

Question 16

When are anti-topoisomerase antibodies often found in systemic sclerosis?

Answer 16

In DCSS and interstitial lung disease

Question 17

When are anti-RNA polymerase antibodies found in systemic sclerosis?

Answer 17

In DCSS

Question 18

What pathway of therapy is taken in management of LCSS?

Answer 18

Vascular therapy
Severe organ-based complication treatment
Common morbidity management

Question 19

What pathway is taken in management of DCSS?

Answer 19

Vascular therapy
Immunosuppressive therapy
Severe organ-based complication treatment
Common morbidity management

Question 20

How is reflux treated in systemic sclerosis?

Answer 20

PPI ± Ranitidine ± Gaviscon

Question 21

How is pulmonary fibrosis treated in systemic sclerosis?

Answer 21

Immunosuppression

Question 22

How is rapidly progressive skin involvement treated in systemic sclerosis?

Answer 22

Immunosuppression

Question 23

How is pulmonary hypertension treated in systemic sclerosis?

Answer 23

Prostaglandin analogues
Endothelial receptor antagonists
PDE5 inhibitors
ACE inhibitors for control