Systemic Sclerosis

Question 1

What is systemic sclerosis characterised by?

Answer 1

A condition of unknown aetiology characterised by hardened, sclerotic skin and other connective tissues.

Question 2

How much more common is systemic sclerosis in females?

Answer 2

It is four times more common in females.

Question 3

What are the three patterns of disease in systemic sclerosis?

Answer 3

Limited cutaneous systemic sclerosis, diffuse cutaneous systemic sclerosis and scleroderma without internal organ involvement.

Question 4

What is often the first sign of limited cutaneous systemic sclerosis?

Answer 4

Raynaud’s phenomenon.

Question 5

Which areas are affected in limited cutaneous systemic sclerosis?

Answer 5

The face and distal limbs.

Question 6

Which antibody is associated with limited cutaneous systemic sclerosis?

Answer 6

Anti-centromere antibodies.

Question 7

What does the CREST acronym stand for?

Answer 7

Calcinosis, Raynaud’s phenomenon, oEsophageal dysmotility, Sclerodactyly and Telangiectasia.

Question 8

Which areas are affected in diffuse cutaneous systemic sclerosis?

Answer 8

The trunk and proximal limbs.

Question 9

Which antibody is associated with diffuse cutaneous systemic sclerosis?

Answer 9

Anti-scl-70 antibodies.

Question 10

What is now the most common cause of death in diffuse cutaneous systemic sclerosis?

Answer 10

Respiratory involvement including interstitial lung disease and pulmonary arterial hypertension.

Question 11

What renal complication may occur in diffuse systemic sclerosis?

Answer 11

Renal disease and hypertension.

Question 12

What medication should be started in renal disease due to systemic sclerosis?

Answer 12

An ACE inhibitor, typically captopril.

Question 13

Why is captopril used for scleroderma renal crisis?

Answer 13

Because of its rapid onset and short half-life allowing dose titration.

Question 14

What mechanism do ACE inhibitors target in scleroderma renal disease?

Answer 14

They reduce efferent arteriolar vasoconstriction and limit renin-angiotensin system activation.

Question 15

What is the general prognosis of diffuse cutaneous systemic sclerosis?

Answer 15

Poor prognosis.

Question 16

What characterises scleroderma without internal organ involvement?

Answer 16

Tightening and fibrosis of the skin.

Question 17

What forms can skin involvement take in localised scleroderma?

Answer 17

Plaques (morphoea) or linear lesions.

Question 18

What percentage of systemic sclerosis patients are ANA positive?

Answer 18

90%.

Question 19

What percentage of systemic sclerosis patients are rheumatoid factor positive?

Answer 19

30%.

Question 20

Which antibody is associated with severe interstitial lung disease in systemic sclerosis?

Answer 20

Anti-scl-70 antibodies.

Question 21

What nailfold capillaroscopy findings suggest systemic sclerosis?

Answer 21

Dilated capillary loops, capillary dropout areas and capillary haemorrhages.

Question 22

Which gastrointestinal manifestation is extremely common in systemic sclerosis?

Answer 22

Oesophageal dysmotility causing dysphagia, GORD and worsening symptoms when lying flat.

Question 23

What causes oesophageal dysmotility in systemic sclerosis?

Answer 23

Fibrosis of smooth muscle leading to poor lower oesophageal sphincter tone.

Question 24

Which form of systemic sclerosis is commonly associated with pulmonary arterial hypertension?

Answer 24

Limited cutaneous systemic sclerosis.

Question 25

What clinical features suggest pulmonary arterial hypertension in systemic sclerosis?

Answer 25

Progressive exertional dyspnoea, loud P2, right ventricular heave and raised JVP.

Question 26

What investigations help diagnose pulmonary arterial hypertension in systemic sclerosis?

Answer 26

Echocardiography and NT-proBNP.

Question 27

Which drug classes are used to manage pulmonary arterial hypertension in systemic sclerosis?

Answer 27

Endothelin receptor antagonists, PDE-5 inhibitors and prostacyclin analogues.

Question 28

Which form of systemic sclerosis is commonly associated with interstitial lung disease?

Answer 28

Diffuse cutaneous systemic sclerosis.

Question 29

What clinical findings suggest interstitial lung disease in systemic sclerosis?

Answer 29

Bibasal fine inspiratory crackles, restrictive lung pattern, reduced DLCO and HRCT showing ground-glass changes or fibrosis.

Question 30

What are the hallmark features of scleroderma renal crisis?

Answer 30

Sudden severe hypertension, rapidly rising creatinine, proteinuria, haematuria and microangiopathic haemolytic anaemia.

Question 31

What medication class can trigger scleroderma renal crisis?

Answer 31

High-dose steroids.

Question 32

What complications can calcinosis cause in systemic sclerosis?

Answer 32

Ulceration, infection and pain.

Question 33

Where do telangiectasia commonly occur in systemic sclerosis?

Answer 33

On the face, lips, hands and oral mucosa.

Question 34

What causes digital ulceration in systemic sclerosis?

Answer 34

Severe Raynaud’s phenomenon and vessel narrowing leading to ischaemia.

Question 35

How is skin involvement distributed in limited cutaneous systemic sclerosis?

Answer 35

Skin thickening distal to the elbows and knees and affecting the face.

Question 36

How is skin involvement distributed in diffuse cutaneous systemic sclerosis?

Answer 36

Skin thickening of the trunk and proximal limbs.

Question 37

What are “salt and pepper” skin changes in systemic sclerosis?

Answer 37

Patchy hypopigmentation with scattered dark spots.