Rheumatology - Systemic Sclerosis

Question 1

What is systemic sclerosis?

Answer 1

Autoimmune connective tissue disease involving inflammation and fibrosis of the connective tissues skin and internal organs

Question 2

What are the main patterns of disease in systemic sclerosis?

Answer 2

Limited cutaneous systemic sclerosis
Diffuse cutaneous systemic sclerosis

Question 3

What mnemonic is used to remember limited cutaneous systemic sclerosis?

Answer 3

CREST
Calcinosis
Raynaud’s phenomenon
oEosphageal dysmotility
Sclerodactyly
Telangiectasia

Question 4

What are the features of diffuse cutaneous systemic sclerosis?

Answer 4

CREST +
Cardiovascular problems (hypertension and coronary artery disease)
Lung problems (pulmonary hypertension and pulmonary fibrosis)
Kidney problems (glomerulonephritis and scleroderma renal crisis)

Question 5

What is scleroderma?

Answer 5

Hardening of the skin

Shiny, tight skin without normal skin folds

Hands and face

Question 6

What is sclerodactyly?

Answer 6

Skin changes in the hands

Skin tightening around joints restricts motion and reduces function, fat pads on fingers are lost

Skin so tight it can break and heals poorly

Question 7

What is telangiectasia?

Answer 7

Dilated blood vessels in the skin

Question 8

What is calcinosis?

Answer 8

Calcium deposits under the skin, most commonly found on the fingertips

Question 9

What is oesophageal dysmotility?

Answer 9

Caused by atrophy and dysfunction of smooth muscle as well as fibrosis of the oesophagus

Question 10

What is scleroderma renal crisis?

Answer 10

Severe hypertension and renal failure

Question 11

What can trigger Raynaud’s phenomenon?

Answer 11

Mild cold triggers e.g. opening the fridge

Question 12

What causes Raynaud’s phenomenon?

Answer 12

Vasoconstriction

Question 13

What is the typical pattern of Raynaud’s phenomenon?

Answer 13

White- vasoconstriction
Blue- cyanosis
Red- Reperfusion and hyperaemia

Question 14

What is Raynaud’s disease?

Answer 14

Where Raynaud’s phenomenon occurs without an associated systemic disease

80-90% of patients with the phenomenon

Question 15

What is nailfold capillaroscopy for?

Answer 15

Magnify and examine peripheral capillaries where skin meets the base of the fingernail

Abnormal capillaries
Avascular areas
Micro-haemorrhages

Suggest systemic sclerosis

Patients with Raynaud’s disease have normal nailfold capillaries

Question 16

What are the treatment options for Raynaud’s?

Answer 16

Keep hands warm
Nifedipine

Beta blockers worsen symptoms

Question 17

What are the autoantibodies in systemic sclerosis?

Answer 17

ANA (non-specific, positive in SLE and autoimmune hepatitis)

Anti-centromere antibodies most associated with limited cutaneous systemic sclerosis

Anti-Scl-70 antibodies most associated with diffuse cutaneous systemic sclerosis

Question 18

How is systemic sclerosis managed?

Answer 18

DMARDs (methotrexate)
Biologic therapies (rituximab)
Steroids - can increase risk of scleroderma renal crisis

Conservative
- Smoking cessation
- Skin stretching
- Emolients
- Avoid cold triggers for Raynaud’s
- Occupational therapy

Medical management for symptoms
- Nifedipine
- PPIs for refulux
- Metoclopramide for GI symptoms
- Antihypertensives